Download - ANEMIA IN PREGNANCY BY DR SHASHWAT JANI
ANAEMIA IN PREGNANCY
Dr SHASHWAT JANI Dr SHASHWAT JANI MS ( OBS - GYN MS ( OBS - GYN ))
Diploma in Advanced laparoscopy Diploma in Advanced laparoscopy Assistant Professor Sheth V S General HospitalAssistant Professor Sheth V S General Hospital
Smt NHL Municipal Medical college AhmedabadSmt NHL Municipal Medical college Ahmedabad
Mobile +91 99099 44160Mobile +91 99099 44160
E- mail drshashwatjanigmailcomE- mail drshashwatjanigmailcom
Commonest haematological dsCommonest haematological ds
40-60 maternal deaths in developing 40-60 maternal deaths in developing countriescountries
Death bo cardiac failure haemorrhage Death bo cardiac failure haemorrhage infection pre-eclampsiainfection pre-eclampsia
Definition Definition
Condition of low circulating Hb in which Condition of low circulating Hb in which Hb concentration has fallen below a Hb concentration has fallen below a threshold lying at 2 standard deviation threshold lying at 2 standard deviation below the median of a healthy population below the median of a healthy population of same age sex stage of pregnancyof same age sex stage of pregnancy
WHO WHO ICMR categorises anaemia depending on ICMR categorises anaemia depending on
Hb levelHb level
Classification of AnaemiaClassification of Anaemia
PhysiologicalPhysiological
PathologicalPathological
Causes of pathological anaemiaCauses of pathological anaemia
Deficiency Deficiency anaemiaanaemia
Haemorrhagic Haemorrhagic anaemia anaemia
Acute chronicAcute chronic
Hereditary Hereditary anaemiaanaemia
Bone marrow Bone marrow insufficiency ndash insufficiency ndash aplasia hypoaplasia hypo
Anaemia of Anaemia of infection- infection- malaria TBmalaria TB
Chronic ds- Chronic ds- renalrenal
Concept of physiological anaemiaConcept of physiological anaemia
Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement
Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation
Iron requirementIron requirement
Menstruating females 1-2mg per dayMenstruating females 1-2mg per day
Pregnant females 15-25mg per dayPregnant females 15-25mg per day
Children 1mg per dayChildren 1mg per day
An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed
Total iron requirement in pregnancyTotal iron requirement in pregnancy
Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday
in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk
Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed
Iron distribution on pregnancyIron distribution on pregnancy
Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg
Iron distribution in bodyIron distribution in body
Total iron 3-5gTotal iron 3-5g
Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g
Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g
Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g
Plasma iron 3-4mgPlasma iron 3-4mg
Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Commonest haematological dsCommonest haematological ds
40-60 maternal deaths in developing 40-60 maternal deaths in developing countriescountries
Death bo cardiac failure haemorrhage Death bo cardiac failure haemorrhage infection pre-eclampsiainfection pre-eclampsia
Definition Definition
Condition of low circulating Hb in which Condition of low circulating Hb in which Hb concentration has fallen below a Hb concentration has fallen below a threshold lying at 2 standard deviation threshold lying at 2 standard deviation below the median of a healthy population below the median of a healthy population of same age sex stage of pregnancyof same age sex stage of pregnancy
WHO WHO ICMR categorises anaemia depending on ICMR categorises anaemia depending on
Hb levelHb level
Classification of AnaemiaClassification of Anaemia
PhysiologicalPhysiological
PathologicalPathological
Causes of pathological anaemiaCauses of pathological anaemia
Deficiency Deficiency anaemiaanaemia
Haemorrhagic Haemorrhagic anaemia anaemia
Acute chronicAcute chronic
Hereditary Hereditary anaemiaanaemia
Bone marrow Bone marrow insufficiency ndash insufficiency ndash aplasia hypoaplasia hypo
Anaemia of Anaemia of infection- infection- malaria TBmalaria TB
Chronic ds- Chronic ds- renalrenal
Concept of physiological anaemiaConcept of physiological anaemia
Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement
Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation
Iron requirementIron requirement
Menstruating females 1-2mg per dayMenstruating females 1-2mg per day
Pregnant females 15-25mg per dayPregnant females 15-25mg per day
Children 1mg per dayChildren 1mg per day
An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed
Total iron requirement in pregnancyTotal iron requirement in pregnancy
Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday
in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk
Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed
Iron distribution on pregnancyIron distribution on pregnancy
Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg
Iron distribution in bodyIron distribution in body
Total iron 3-5gTotal iron 3-5g
Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g
Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g
Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g
Plasma iron 3-4mgPlasma iron 3-4mg
Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Definition Definition
Condition of low circulating Hb in which Condition of low circulating Hb in which Hb concentration has fallen below a Hb concentration has fallen below a threshold lying at 2 standard deviation threshold lying at 2 standard deviation below the median of a healthy population below the median of a healthy population of same age sex stage of pregnancyof same age sex stage of pregnancy
WHO WHO ICMR categorises anaemia depending on ICMR categorises anaemia depending on
Hb levelHb level
Classification of AnaemiaClassification of Anaemia
PhysiologicalPhysiological
PathologicalPathological
Causes of pathological anaemiaCauses of pathological anaemia
Deficiency Deficiency anaemiaanaemia
Haemorrhagic Haemorrhagic anaemia anaemia
Acute chronicAcute chronic
Hereditary Hereditary anaemiaanaemia
Bone marrow Bone marrow insufficiency ndash insufficiency ndash aplasia hypoaplasia hypo
Anaemia of Anaemia of infection- infection- malaria TBmalaria TB
Chronic ds- Chronic ds- renalrenal
Concept of physiological anaemiaConcept of physiological anaemia
Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement
Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation
Iron requirementIron requirement
Menstruating females 1-2mg per dayMenstruating females 1-2mg per day
Pregnant females 15-25mg per dayPregnant females 15-25mg per day
Children 1mg per dayChildren 1mg per day
An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed
Total iron requirement in pregnancyTotal iron requirement in pregnancy
Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday
in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk
Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed
Iron distribution on pregnancyIron distribution on pregnancy
Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg
Iron distribution in bodyIron distribution in body
Total iron 3-5gTotal iron 3-5g
Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g
Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g
Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g
Plasma iron 3-4mgPlasma iron 3-4mg
Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Classification of AnaemiaClassification of Anaemia
PhysiologicalPhysiological
PathologicalPathological
Causes of pathological anaemiaCauses of pathological anaemia
Deficiency Deficiency anaemiaanaemia
Haemorrhagic Haemorrhagic anaemia anaemia
Acute chronicAcute chronic
Hereditary Hereditary anaemiaanaemia
Bone marrow Bone marrow insufficiency ndash insufficiency ndash aplasia hypoaplasia hypo
Anaemia of Anaemia of infection- infection- malaria TBmalaria TB
Chronic ds- Chronic ds- renalrenal
Concept of physiological anaemiaConcept of physiological anaemia
Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement
Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation
Iron requirementIron requirement
Menstruating females 1-2mg per dayMenstruating females 1-2mg per day
Pregnant females 15-25mg per dayPregnant females 15-25mg per day
Children 1mg per dayChildren 1mg per day
An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed
Total iron requirement in pregnancyTotal iron requirement in pregnancy
Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday
in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk
Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed
Iron distribution on pregnancyIron distribution on pregnancy
Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg
Iron distribution in bodyIron distribution in body
Total iron 3-5gTotal iron 3-5g
Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g
Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g
Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g
Plasma iron 3-4mgPlasma iron 3-4mg
Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Causes of pathological anaemiaCauses of pathological anaemia
Deficiency Deficiency anaemiaanaemia
Haemorrhagic Haemorrhagic anaemia anaemia
Acute chronicAcute chronic
Hereditary Hereditary anaemiaanaemia
Bone marrow Bone marrow insufficiency ndash insufficiency ndash aplasia hypoaplasia hypo
Anaemia of Anaemia of infection- infection- malaria TBmalaria TB
Chronic ds- Chronic ds- renalrenal
Concept of physiological anaemiaConcept of physiological anaemia
Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement
Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation
Iron requirementIron requirement
Menstruating females 1-2mg per dayMenstruating females 1-2mg per day
Pregnant females 15-25mg per dayPregnant females 15-25mg per day
Children 1mg per dayChildren 1mg per day
An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed
Total iron requirement in pregnancyTotal iron requirement in pregnancy
Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday
in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk
Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed
Iron distribution on pregnancyIron distribution on pregnancy
Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg
Iron distribution in bodyIron distribution in body
Total iron 3-5gTotal iron 3-5g
Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g
Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g
Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g
Plasma iron 3-4mgPlasma iron 3-4mg
Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Concept of physiological anaemiaConcept of physiological anaemia
Increase Increase bullPlasma volumePlasma volumebullTIBCTIBCbullIRON requirementIRON requirement
Decrease Decrease bullHbHbbullRed cellRed cellbullPCVPCVbullMCVMCVbullMCHMCHbullMCHCMCHCbullSIronSIronbullSaturationSaturation
Iron requirementIron requirement
Menstruating females 1-2mg per dayMenstruating females 1-2mg per day
Pregnant females 15-25mg per dayPregnant females 15-25mg per day
Children 1mg per dayChildren 1mg per day
An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed
Total iron requirement in pregnancyTotal iron requirement in pregnancy
Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday
in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk
Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed
Iron distribution on pregnancyIron distribution on pregnancy
Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg
Iron distribution in bodyIron distribution in body
Total iron 3-5gTotal iron 3-5g
Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g
Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g
Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g
Plasma iron 3-4mgPlasma iron 3-4mg
Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Iron requirementIron requirement
Menstruating females 1-2mg per dayMenstruating females 1-2mg per day
Pregnant females 15-25mg per dayPregnant females 15-25mg per day
Children 1mg per dayChildren 1mg per day
An adequate diet contains 15mg of iron 10 of An adequate diet contains 15mg of iron 10 of which is absorbedwhich is absorbed
Total iron requirement in pregnancyTotal iron requirement in pregnancy
Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday
in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk
Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed
Iron distribution on pregnancyIron distribution on pregnancy
Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg
Iron distribution in bodyIron distribution in body
Total iron 3-5gTotal iron 3-5g
Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g
Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g
Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g
Plasma iron 3-4mgPlasma iron 3-4mg
Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Total iron requirement in pregnancyTotal iron requirement in pregnancy
Total requirement 700-1400mgTotal requirement 700-1400mg Average 4mgday increases to 66mgday Average 4mgday increases to 66mgday
in last few weeksin last few weeks 25mgd early 125mgd early 1stst trimester trimester 55mgd 20-32wk55mgd 20-32wk 6-8mgd gt32wk6-8mgd gt32wk
Diet provides 14mg iron (5-15) Diet provides 14mg iron (5-15) absorbed ie 1-2mg absorbedabsorbed ie 1-2mg absorbed
Iron distribution on pregnancyIron distribution on pregnancy
Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg
Iron distribution in bodyIron distribution in body
Total iron 3-5gTotal iron 3-5g
Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g
Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g
Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g
Plasma iron 3-4mgPlasma iron 3-4mg
Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Iron distribution on pregnancyIron distribution on pregnancy
Basal iron 280mgBasal iron 280mg Expansion red cell mass 570mgExpansion red cell mass 570mg Fetal transfer 200-350mgFetal transfer 200-350mg Placenta 50-150mgPlacenta 50-150mg Blood loss 100-250mgBlood loss 100-250mg
Iron distribution in bodyIron distribution in body
Total iron 3-5gTotal iron 3-5g
Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g
Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g
Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g
Plasma iron 3-4mgPlasma iron 3-4mg
Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Iron distribution in bodyIron distribution in body
Total iron 3-5gTotal iron 3-5g
Iron in Hb 23Iron in Hb 23rdrd of total iron ie 23 - 33g of total iron ie 23 - 33g
Storage iron (ferritin hemosidrin) 1gStorage iron (ferritin hemosidrin) 1g
Essential iron (myoglobin enzymes) 05gEssential iron (myoglobin enzymes) 05g
Plasma iron 3-4mgPlasma iron 3-4mg
Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
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Iron absorptionIron absorption Inorganic ironInorganic iron((dietary iron ferric form)dietary iron ferric form)
Cereals seeds veg milk eggCereals seeds veg milk egg
Abs uarr by enhancers haem Abs uarr by enhancers haem protein ascorbic acid alcoholprotein ascorbic acid alcohol
Abs darr by inhibitors fibre Abs darr by inhibitors fibre phytic acid calcium tannin phytic acid calcium tannin tea coffee chocolatetea coffee chocolate
Absorption varies 2-100Absorption varies 2-100
Haem ironHaem iron((ferrous form)ferrous form)Derived from Hb Derived from Hb
myoglobinmyoglobin
Animal blood flesh visceraAnimal blood flesh viscera N Absorption 15-30N Absorption 15-30
Iron deficiency 50-90Iron deficiency 50-90
Not affected by inhibitorsNot affected by inhibitors
Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
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Erythropoiesis Erythropoiesis
For erythropoiesis minerals vitamins For erythropoiesis minerals vitamins protein hormones are neededprotein hormones are needed
Minerals iron copper cobaltMinerals iron copper cobalt Protein erythropoietinProtein erythropoietin Vitamins folic acid vit B12 vit C vit B6 Vitamins folic acid vit B12 vit C vit B6
riboflavinriboflavin Hormones androgens thyroxinHormones androgens thyroxin
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Causes of iron deficiency anaemiaCauses of iron deficiency anaemia
Faulty dietary habitsFaulty dietary habits Defective iron absorption dt intestinal infDefective iron absorption dt intestinal inf uarr uarr iron lossiron loss uarr uarr iron demandiron demand darr darr intake of ironintake of iron Pre-pregnancy health statusPre-pregnancy health status
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Clinical Features Clinical Features
SS are dt anaemia itself or ds causingSS are dt anaemia itself or ds causing Symptoms Symptoms fatigue weakness lassitudefatigue weakness lassitude muscle muscle
weakness palpitation syncope angina dyspnoea weakness palpitation syncope angina dyspnoea pedal edemapedal edema
Signs Signs pallor edema nail changes tachycardia pallor edema nail changes tachycardia uarrpulse pressureuarrpulse pressure
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Systemic changesSystemic changes
CVS CVS Effect of hypoxia on heart high cardiac output state Effect of hypoxia on heart high cardiac output state
(dyspnoea on exertion is common in severe anaemia ndashon (dyspnoea on exertion is common in severe anaemia ndashon rest)rest)
Haemic murmur ndashmid systole murmurHaemic murmur ndashmid systole murmur
ventricular dilatationventricular dilatation
heart failureheart failure
Very severe anaemia can ppt heart failure in pt with normal heartVery severe anaemia can ppt heart failure in pt with normal heart
ECG ECG changes may occur with Hb lt6gdlchanges may occur with Hb lt6gdl
normal QRS Wavenormal QRS Wave
depression of ST segmentdepression of ST segment
Flattening inversion of T Wave Flattening inversion of T Wave
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
bull CNSCNS Faintness giddiness headache tinnitus lack Faintness giddiness headache tinnitus lack of concentration drowsiness tingling numbnessof concentration drowsiness tingling numbness
bull Eyes Eyes pallor retina ndashpale pallor retina ndashpale
papillodema ndashrare papillodema ndashrare
bull Reproductive Reproductive menstrual disturbance (non ndashmenstrual disturbance (non ndashpregnant )pregnant )
bull Renal disturbance Renal disturbance slight proteinuriaslight proteinuria
bull GIT GIT anorexia glossitis stomatitis constipation anorexia glossitis stomatitis constipation
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Effect of anaemia on pregnancyEffect of anaemia on pregnancy
Mild anaemia Mild anaemia
No effect except No effect except darriron storedarriron store
Moderate Moderate anaemiaanaemiabulluarruarrweaknessweaknessbullLack of energy Lack of energy FatigueFatiguebullPoor work Poor work performanceperformance
Severe Severe anaemiaanaemiabullPoor outcomePoor outcomebullPalpitation Palpitation bullTachycardia Tachycardia bullBreathlessnessBreathlessnessbullCardiac failureCardiac failure
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Investigation Investigation darrdarrHb conc is late featureHb conc is late feature
Red cell indicesRed cell indices
Non pregnant -darrMCV is earliest featureNon pregnant -darrMCV is earliest feature
Pregnancy ndashMCV is not sensitive Pregnancy ndashMCV is not sensitive indicatorindicator
darr darrMCVMCV
darr darrMCHMCH
darr darrMCHCMCHC
SFerritinSFerritin stored iron stored iron
normal 15-300normal 15-300μμglgl
lt12lt12μμgL indicate iron deficiencygL indicate iron deficiency
not affected by recent iron not affected by recent iron ingestioningestion
SIronSIron SIron lt60 SIron lt60μμgdl (60-120gdl (60-120μμgdl)gdl)
TIBC gt350TIBC gt350μμgdl (300-gdl (300-350350μμgdl)gdl)
Transferrin sat lt15 (30)Transferrin sat lt15 (30)
Indicate iron deficiency anaemiaIndicate iron deficiency anaemia
Free erythrocyte protoporphyrin uarrFree erythrocyte protoporphyrin uarr
Serum transferrin receptorSerum transferrin receptor
sensitive and specific markersensitive and specific marker
cellular iron statuscellular iron status
level uarr in cellular iron deficiencylevel uarr in cellular iron deficiency
Bone marrowBone marrow no response to therapy after 4 no response to therapy after 4 wkwk
diagnosis of aplastic anaemiadiagnosis of aplastic anaemia
kala azarkala azar
Depleted store darrferritinDepleted store darrferritin
Iron deficiency with no anaemia Iron deficiency with no anaemia
darr darr ferritinferritin
darr darr transferrin sattransferrin sat
uarr uarrFEPFEP
Anaemia with iron deficiency Anaemia with iron deficiency
darr darrHb red cell indicesHb red cell indices
darr darr transferrin sattransferrin sat
uarr uarrTIBC FEPTIBC FEP
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Normal Blood FilmNormal Blood Film
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
MICROCYTESMICROCYTES
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
HYPOCHROMIAHYPOCHROMIA
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
COMPLICATIONCOMPLICATION
During During PregnancyPregnancybullPre-eclampsia Pre-eclampsia bullIntercurrent Intercurrent infectioninfectionbullHeart failureHeart failurebullPreterm Preterm deliverydelivery
Labour Labour
bullPPHPPHbullHeart failureHeart failurebullShock Shock bullDystocia Dystocia
PuerperiumPuerperium
bullPsepsis Psepsis bullSub involutionSub involutionbullFailing Failing lactationlactationbullP venous P venous thrombosisthrombosis
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Risk period Risk period 30-32wk pregnancy30-32wk pregnancy
During laborDuring labor
Immediately following deliveryImmediately following delivery
Puerperium (7-10days)Puerperium (7-10days) Fetal complication Fetal complication LBWLBW
IUDIUD
Preterm babyPreterm baby
SGASGA
uarr uarrPerinatal morbidityamp mortalityPerinatal morbidityamp mortality
IUGRIUGR
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
TREATMENTTREATMENT
PROPHYLACTICPROPHYLACTIC --Avoidance of frequent child birthAvoidance of frequent child birth
-Dietary prescription-Dietary prescription
-Food fortification-Food fortification
-Supplement iron therapy-Supplement iron therapy
100mg elemental iron 100mg elemental iron + 500+ 500μμg folate forg folate for
minimum 100 Daysminimum 100 Days
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Curative Curative
bull Choice of therapy depend onChoice of therapy depend onbull Duration of pregnancyDuration of pregnancybull Severity of anaemiaSeverity of anaemiabull Associated complicated factorAssociated complicated factor
bull Iron therapyIron therapy --oral oral
- parentral- parentral
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
WHOWHO 30-60 mgd normal iron store30-60 mgd normal iron store
120-240mgd no iron store120-240mgd no iron store
Oral ironOral iron Elemental doseElemental dose
Ferrous sulphateFerrous sulphate 6565
FSulphate(exsiccated)FSulphate(exsiccated) 6565
Ferrous gluconateFerrous gluconate 3636
Ferrous fumarateFerrous fumarate 6767
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Ferrous sulphateFerrous sulphate is cheap is cheap Rest are expansive but have less epigastric discomfort Rest are expansive but have less epigastric discomfort
nausea vomitingnausea vomiting
Slow release preparationSlow release preparation expansive no SE expansive no SE
Much iron not released unabsorbed excreted Much iron not released unabsorbed excreted unchangedunchanged
uarr uarrdose required to achieve given responsedose required to achieve given response
Rate of improvement Rate of improvement evident in 3wk Hb rise 07-1gdlwkevident in 3wk Hb rise 07-1gdlwk
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Drawback Drawback Intolerance Intolerance
unpredictable abs rateunpredictable abs rate
Siron restored but difficult to replenish Siron restored but difficult to replenish
iron storeiron store Response to therapyResponse to therapy sense of well being sense of well being
uarr uarrAppetiteAppetite
Improved outlookImproved outlook
haematologically improvhaematologically improv Causes of failure Causes of failure Improper typing def absorptionImproper typing def absorption
failure to take iron infectionfailure to take iron infection
conc Blood loss folate def conc Blood loss folate def
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Parentral iron therapyParentral iron therapy
IVIV IMIM Dose Dose 03 03 x wt (lb) (100-Hb)x wt (lb) (100-Hb) (Hb ndash pt Hb) (Hb ndash pt Hb) x wt (kg) x221+1000x wt (kg) x221+1000 250mg250mg elemental iron for each g below N elemental iron for each g below N
IntravenousIntravenous eliminate repeated amp painful IM injeliminate repeated amp painful IM inj
Tt completed in a dayTt completed in a day
TDI amp Undiluted TDI amp Undiluted
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
TDITDI- - Diluted in a lt of isotonic saline or 5 dextroseDiluted in a lt of isotonic saline or 5 dextrose
-Maximum conc used -Maximum conc used 25gltd 25gltd dilutent dilutent
-15 drops min then 45-60 dropsmin (over in -15 drops min then 45-60 dropsmin (over in 4- 5hr)4- 5hr)
SESE chills darrBP chest pain rigor hemolysis chills darrBP chest pain rigor hemolysis anaphylactic reactionanaphylactic reaction
Stop infusionStop infusion anti H steroids adrenalineanti H steroids adrenaline
Undiluted preparationUndiluted preparation given in single dosegiven in single dose
Test dose 05ml iron dextran diluted with 4-5ml of pt Test dose 05ml iron dextran diluted with 4-5ml of pt blood given slowlyblood given slowly
Therapeutic doseTherapeutic dose up to 5ml slow 1mlminup to 5ml slow 1mlmin
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Intramuscular Intramuscular Iron dextran Iron dextran colloid of ferric hydroxide with dextran colloid of ferric hydroxide with dextran
Inj Site should never be massagedInj Site should never be massaged Iron sorbitol citrate Iron sorbitol citrate small molecule rapidly small molecule rapidly
cleared from bodycleared from body
30excreted unchanged30excreted unchanged
SE SE Headache nausea vomiting if given Headache nausea vomiting if given simultaneously with ironsimultaneously with iron
DOSE DOSE 15 mgkg15 mgkg Parentral iron Parentral iron CICI HO Allergy ndashasthma HO Allergy ndashasthma
HO previous reactionHO previous reaction
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Iron Dextran Adverse Reactions
1048721Dose related arthralgia backache chillsdizziness moderate to high feverheadache malaise myalgia
1048721Increased incidence of these effects withtotal dose infusions
1048721Onset is 24-48 hours after administration
1048721Effects subside within 3-4 days
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Sodium Ferric Gluconate(Ferrlicit) Dose
1048721Give 10ml (125mg elemental iron) during
a total of 8 consecutive dialysis sessions
for a total dose of 1000mg elemental iron
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Sodium Ferric GluconateAdministration
1048721Dilute 2ml test dose (25mg elemental iron) in 50ml normal saline and give over 60 minutes
1048721Dilute 10ml (125mg elemental iron) in 100ml normal saline and give over 60 minutes (21mgmin)
1048721Undiluted as a slow IV injection at a rate up to 125mgmin
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Adverse Reactions1048721Hypotensionflushing1048721Associated with rapid administration1048721Not associated with hypersensitivity reactions1048721Resolves within 1-2 hours
1048721Symptoms abdominal pain diarrhea vomiting leading to pallor or cyanosis lassitude drowsiness hyperventilation due to acidosis amp CV collapse
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Iron Sucrose (Venofer) Dose1048721
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Iron Sucrose (Venofer)Monitoring1048721Hematologic and hematinic factorsndash Hb Hct serum ferritin transferrin saturationndash because transferrin values increase rapidly after IV administration serum iron values may be reliably obtained 48 hours after dosing
1048721Experienced in gt 5 of patientsndash hypotensionndash crampsleg crampsndash nauseandash headachendash vomitingndash diarrhea
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
If no time to achieve reasonable Hb concIf no time to achieve reasonable Hb conc
BLOOD TRANSFUSIONBLOOD TRANSFUSION PACKED RED CELL PREFERREDPACKED RED CELL PREFERRED
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
ANC CARE
lt30WK 30-36WK gt36WK
IRON DEF FOLIC ACID DEF
ORAL IRON
INTOLERANCENON COMLIANCE
IM IV
FOLIC ACID
IRON DEF FOLIC ACID DEF
PARENTRAL
IMIV
ORAL FOLATE
BLOOD TRANSFUSION
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
MANAGEMENT IN LABORMANAGEMENT IN LABOR 11stst stage - stage - comfortable positioncomfortable position
- pain relief- pain relief -oxygen ready-oxygen ready -digitalisation may be required-digitalisation may be required -avoid drugs causing tachycardia-avoid drugs causing tachycardia -antibiotic prophylaxis-antibiotic prophylaxis -blood to kept ready (cross -blood to kept ready (cross
match)match)
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
2nd stage 2nd stage more stressfulmore stressful -uarr chance of CCF-uarr chance of CCF - shorten by forceps vacuum- shorten by forceps vacuum
3rd stage 3rd stage active managementactive management
4th stage 4th stage stage of monitoring PPHstage of monitoring PPH treated energitically as these treated energitically as these pt tolerate bleeding poorlypt tolerate bleeding poorly
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Megaloblastic anaemiaMegaloblastic anaemia
Derangement in RBC maturation with Derangement in RBC maturation with production in BM of abn Precursor kas production in BM of abn Precursor kas megaloblast dt impaired DNA synthesismegaloblast dt impaired DNA synthesis
Deficiency of folate vitamin B12 or bothDeficiency of folate vitamin B12 or both
Vit B12 def rare in pregnancyVit B12 def rare in pregnancy
Anaemia nearly bo Anaemia nearly bo folic acidfolic acid def def
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Causes of Folic acid deficiencyCauses of Folic acid deficiency
bull Inadequate intakeInadequate intake nausea anorexianausea anorexia
dietary insufficiencydietary insufficiency excess cooking destroys FAexcess cooking destroys FA AlcholicAlcholicbull Increased demandIncreased demand pregnancy ( 400pregnancy ( 400μμg)g) lactationlactation
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
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YOU
darr darr AbsorptionAbsorption
intestinal absorption syndromeintestinal absorption syndrome Abnormal demandAbnormal demand
twin pregnancytwin pregnancy infectioninfection haemorraghic statushaemorraghic status Failure of utilisation Failure of utilisation anticonvulsant therapyanticonvulsant therapy infectioninfection darr darr storagestorage
liver dsliver ds vit C defvit C def
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Folic acid absorbed in duodenum jejunumFolic acid absorbed in duodenum jejunum Function Function methylation of homocysteine to methylation of homocysteine to
methiononemethionone synthesis of pyrimidinesynthesis of pyrimidine
Incidence 05- 3Incidence 05- 3 uarr uarr in multiparain multipara
uarr uarrmultiple pregnancymultiple pregnancy
Plasma folate falls as pregnancy advances Plasma folate falls as pregnancy advances becomes frac12 of non pregnant valuebecomes frac12 of non pregnant value
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
VITAMIN BVITAMIN B1212 DEFICIENCY DEFICIENCY
Synthesis by certain micro-organismsSynthesis by certain micro-organisms 2 active metabolic forms2 active metabolic forms
methycobalaminemethycobalamine
adenosylcobalamineadenosylcobalamine Absorption occur in Distal duodenumAbsorption occur in Distal duodenum Dietary source Dietary source
meat fish egg milkmeat fish egg milk Not destroyed by cookingNot destroyed by cooking
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Clinical features in megaloblastic Clinical features in megaloblastic anaemiaanaemia
Insidious last trimester or puerperiumInsidious last trimester or puerperium Anorexia vomitingAnorexia vomiting Unexplained feverrsquoUnexplained feverrsquo Glossitis Haemorraghic patch (skin)Glossitis Haemorraghic patch (skin) Ss of pre-eclampsiaSs of pre-eclampsia Numbness or paresthesia in extremityNumbness or paresthesia in extremity
(vit B12 def) (vit B12 def)
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Investigation of megaloblastic anaemiaInvestigation of megaloblastic anaemia Red cell indices Red cell indices Hb lt10gdlHb lt10gdl
MCV gt96flMCV gt96fl
MCH gt33pgMCH gt33pg
MCHC normalMCHC normal
Sr enzyme Methymalonic acid amp homocysteine Sr enzyme Methymalonic acid amp homocysteine level are raised in Vit B12 def in folic acid def level are raised in Vit B12 def in folic acid def only homocysteine is uarronly homocysteine is uarr
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Plasma folate limited diagnostic valuePlasma folate limited diagnostic value Red cell folate body tissue levelRed cell folate body tissue level uarruarrFIGLU excretion in urineFIGLU excretion in urine darrdarrSr folate (lt3ngdl) amp red cell folate Sr folate (lt3ngdl) amp red cell folate
(lt150ngdl) diagnostic of folic acid (lt150ngdl) diagnostic of folic acid deficiencydeficiency
PS macrocyte hypersegmented PS macrocyte hypersegmented neutrophil neutropenia thrombocytopenianeutrophil neutropenia thrombocytopenia
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Macrocytic anemiaMacrocytic anemia
The RBC are almost as large as the lymphocyte Note the hypersegmented neurotrophil There are fewer RBCs
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Effect of folic acid deficiencyEffect of folic acid deficiency
Effect on pregnancyEffect on pregnancybullAbortionAbortionbullGrowth retardationGrowth retardationbullAbruptio placentaAbruptio placentabullPre -eclampsiaPre -eclampsia
Effect on fetusEffect on fetusbullNeural tube defectNeural tube defectbullAbortionAbortionbullSGASGAbullPre termPre term
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Treatment Treatment Prophylactic Prophylactic Dietary habitDietary habit - avoid over-cooking - avoid over-cooking -food fortification-food fortification WHO Pregnancy 800WHO Pregnancy 800μμgg Lactation 600Lactation 600μμgg Non pregnant 400Non pregnant 400μμgg Treatment Treatment Folic acidFolic acid 5mg oral d till 4 wk 5mg oral d till 4 wk in puerperiumin puerperium Parenteral cyanocobalamine 250Parenteral cyanocobalamine 250μμg g
im every monthim every month
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Haemoglobinopathies Haemoglobinopathies
Defect in synthesis of globin chain of HbDefect in synthesis of globin chain of Hb Eg Thalassemia sickle cell anaemiaEg Thalassemia sickle cell anaemia
Hb E Hb DHb E Hb D
DIAGNOSIS DIAGNOSIS
Hb electrophoresisHb electrophoresis
Molecular techniqueMolecular technique
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
SICKLE CELL ANAEMIASICKLE CELL ANAEMIA
Structural abn Of globin synthesisStructural abn Of globin synthesis AA substitution at 6AA substitution at 6thth position glutamate position glutamate
replacing valinereplacing valine Sickling favouredSickling favoured byby acidosis dehydration coolingacidosis dehydration cooling
Sickle ds
HOMOZYGOUSHbSS
HETEROZYGOUSHbAS
HbSC
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Hb CHb CbullLYSINE for GLUTAMIC LYSINE for GLUTAMIC ACIDACIDbullLess solubleLess solublebullHaemolytic anaemiaHaemolytic anaemiabullNormal near normal HbNormal near normal HbbullUndiagnosed but Undiagnosed but plusmn massive plusmn massive sickling in pregnancysickling in pregnancy
Hb ASHb ASbullNo detectable abnNo detectable abnbullNot anaemic under Not anaemic under additional pregnancy stressadditional pregnancy stressbullCrisis in extreme conditionCrisis in extreme conditionbulluarr uarr incidence pre eclampsiaincidence pre eclampsia
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
DiagnosisDiagnosis Refractory hypochromic anaemia Refractory hypochromic anaemia
-Identified by sickling test-Identified by sickling test
-Persistent leucocytosis-Persistent leucocytosis
-uarrSIron-uarrSIron
- Electrophoresis- Electrophoresis
Effect on pregnancyEffect on pregnancybull Abortion Abortion bull Prematurity Prematurity bull Fetal loss (placental Fetal loss (placental
infarct)infarct)bull Pre eclampsiaPre eclampsiabull PPHPPHbull InfectionInfection
uarruarrMaternal morbidityMaternal morbiditybullInfection Infection bullCardiovascular stressCardiovascular stressbullSickle cell crisesSickle cell crisesbullCerebrovascular Cerebrovascular bullPul InfarctPul InfarctbullEmbolismEmbolism
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
MANAGEMENTMANAGEMENT
role of BM Transfusionrole of BM Transfusion Induction of HbF - Induction of HbF - HydroxyureaHydroxyurea
- Pulse butyrate therapy- Pulse butyrate therapy Labor -Labor -Adequate Hydration Adequate Hydration
-Avoid Hypoxia-Avoid Hypoxia Post natal -Post natal -AntibioticAntibiotic
-Hydration-Hydration
-Contraception (avoid IUCD)-Contraception (avoid IUCD)
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
Thalassemia Thalassemia
Common genetic disorderCommon genetic disorder
darrdarrrate of globin synthesisrate of globin synthesis
Two type Two type
αα Thalassemia Thalassemia
ββ Thalassemia Thalassemia
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
αα Thalassemia Thalassemia Dt gene deletionDt gene deletion 4 types 4 types
1gene mut1gene mutbullNo clinical labNo clinical lab
abn abn bullSilent carrierSilent carrier
2 gene mut2 gene mutbullαα thalassemia thalassemia
minorminorbullUnrecognised Unrecognised
3 gene mut3 gene mutbullHbHHbHbullOne functional One functional
GeneGenebullHb unstable dtHb unstable dt
ββ44 tetramer or tetramer or Hb Bart( Hb Bart(γγ44) ) bullHaemolytic Haemolytic anaemiaanaemia
All 4 geneAll 4 genebullNO NO functional functional
genegenebullIncompatible Incompatible
with lifewith lifebullHydropic Hydropic fetusfetusbullSevere pre Severe pre eclampsiaeclampsia
Abn Red cell indices darrMCV MCH MCHCAbn Red cell indices darrMCV MCH MCHC NormalNormal
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
THANK
YOU
YOU
ββ thalassemia thalassemia
ββ thalassemia major thalassemia major (Cooley anaemia)(Cooley anaemia)
homozygoushomozygous
Erythropoietin ineffectiveErythropoietin ineffective
HepatospleenomegalyHepatospleenomegaly ββ thalassemia minor thalassemia minor heterozygousheterozygous Hb darr NHb darr N Hb AHb A22( ( αα22ζζ2) Hb F2) Hb F
MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
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MANAGEMENTMANAGEMENT
αα Thalassemia ThalassemiabullIron amp folate ANCIron amp folate ANCbullParentral iron CIParentral iron CIbullIF Hb not adequate at term IF Hb not adequate at term
blood transfusionblood transfusionbullPt with HbH ndashN lifePt with HbH ndashN life
expectancy daily folate expectancy daily folate
suppl pregnancy- 5mgdsuppl pregnancy- 5mgd
ββ thalassemia thalassemiaMajor folate supplMajor folate suppl
Iron CIIron CI
Minor oral Iron amp folateMinor oral Iron amp folate
parentral iron CIparentral iron CI
THANK
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YOU
YOU
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YOU
YOU