hope 2013 rmmch- vigneshwar
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Pyrexia of unknown origin-An unusual cause
N.S.VIGNESHWAR
RMMH
Anna!alai "ni#ersi$y
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• 10 months old male infant 2nd born to
nonconsanguineous parents residing at
Sirkali was brought to casualty by hismother whose reliability was good withpresenting complaints of
Fever x 1 monthCough x 3 wks
Abdominal distension x 1 wk
Breathing diculty x 3wks
efusal of feeds x 1! days
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Fever
• Insidious onset• 1 month• low grade Fever , "ntermittent
• No chills or rigor• No H/o rash•
No aggravating factors• Relieved by antipyretics
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oug%
• Cough present on # o$ throughoutthe day disturbing the slee%
• wks duration! Insidious onset• &iculty in breathing " wk• #ore during lying down posture• No H/o foreign body/eye
congestion/mass descending perrectum
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• H/o refusal of fee&s " 1$ days• H/o weig%$ loss present• A'&o!inal &is$ension x (wk
•
No H/o melena% vomiting% loosestools % &aundice% bleeding 'R• No H/o fore head sweating% suck
rest suck cycle %(yanosis• No H/o sei)ures% loss of
consciousness
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A&!ission a$ )!on$%sof age for)* &ays -+R,I
A&!ission a$ !on$%sof agefor (!on$% +R,I wi$%
a'&o!inal&is$ension
A&!i$$e& a$ /!on$%sof agefor 0weeks-+R,Iwi$%a'&o!inal&is$ension
• 1loo& $ransfusion - ane!ia• 2x as 'B # A'' started ( )th mon * no
contact H3o of ,1
AS, HIS,4R5
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B"'+ +",'-./(
• *ntenatal +No H/o ,-R(HIN.(,I-N
• No other maternal risk factors•
Natal + ,erm normal delivery! 0wt 2$kg• 'ostnatal + 3neventful
• 3mbilical cord fell on day 4• 'assed #econium on day 1
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"2"A'"-2 /(
– 0(5 6Scar present7 – -'8 9%1%2% – :', 1%2% – Hep;0 1%2% – Hib 1%2%
• <et to be Immuni)ed with measles2'"'"-2/(
• 0.;= months then started weaning
Ac$ual Ex6ec$e&
Ga6
(alorie 4$6>cal
7
?996>cal
7
)789c
al:
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&4546-742'A6 age appropriate forchronological age
FA"6. +",'-. no H/o similar illness infamily members
,-C"-4C-2-"C ,'A', - 5rade I86*ccording to modiAed >uppusamy scale 7•(attle present
92
4
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+4A& '- F--' 48A"2A'"-2/(
• *. open B Cat• Eyes Pallor ;
• No evidence of 8it deAciency• ars %-ral cavity normal•
.undus ; normal• ars no discharge• Neck ; no lymphadenopathy!• Dimbs 63D/DD7; normal• Pan&igi$al clu''ing ;
• A'&o!en &is$en&e&
• "ternal genitalia/Spine B(ranium ; normal
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AN,HR4P4ME,R5A,"A+ E<PE,E
2
EI5H, F$ >g @$ >g => ?0r& cen$ilegra&e IV8IAP7
Dength ==cm 42cm @1G
H*:(IR(3#.
RN(
F$cm rd to 19th centile
9ellcome trust :#arasmus
9aterlow (1; stunting
Chronic malnutrition
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VI,A+S
• (onscious % alert• ,emp;192 .•
HR;1$2/min! 0';@9/=9 mmhg! (.,secs! RR;42/min ! Sp92;@G• No e"ternal markers of ,0•
No signs of liver cell failure
RESPIRA,4R5 S5S,EM
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RESPIRA,4R5 S5S,EM
INSPE,I4N@-• (hest shape B
movement symmetrical
•
*pical impulsevisible medial to theD, nipple
• No scar% sinuses
• SR;IR; Alanasi <aring =
• No bonyabnormalities
PA+PA,I4N@-
• ,rachea midline• *pical impulse; Dt
Fth intercostal
space J inchmedial to #(D
• 5ocal fremitus
( rightaxillary *infraaxillary #
mammary
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PER"SSI4N +;• &ullness over t axillary * infra
axillary # mammary region
A"S"+,A,I4N+;•
0reath sounds ; bronchial breathsound K R$ axillary infra axillaryB !a!!ary
•
5ocal resonance increased on Rta"illary % infra a"illary B mammary• *dded sounds ; B>6 coarse
cre%titations present in all areasR,LD,
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E<AMINA,I4N
INSPE,I4N@-• :istended• 3mbilicus everted/
displaced down• *ll Muadrants moves
eMually with respiration
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PER"SSI4N@-
• +i#er s6an-(*.7c! 6normal=7
•
No ShiftingdullnessA"S"+,A,I4N@-
•
0S;4/min• No bruit
PA+PA,I4N @-
•No rigidity•+i#er 6al6a'le - c! 'elowRM
–
rounded edges –Surface ; smooth
–.irm in (onsistency
–No 'ulsations
•S6leen 6al6a'le - 0c! +M•No Cuid thrill
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4,HER S5S,EMSVS @-•
S 1 % S 2 heard• No murmurs• No added sounds
NS@-• Higher mental functions intact• (ranial nerves intact on Rt B Dt•
No sensory disturbances• #otor system ; intact• No meningeal signs
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S5MP,4MS BCIN2INGS
- A S"MMAR5
PA++4R
PAN 2IGI,A++"11ING
5MP,4MS@-CEVER4"GH1REA,H+ESSNESS
A124MINA+ &is$ension
,4SP+EN4MEGA+5
RS• VCVR• 1R4NHIA+
1S• 1D+ 4ARSE
REPI,A,I4NS
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• 'yre"ia of unknown origin• Rt side consolidation%
hepatosplenomegaly % with grade I8'# most probably due totuberculosis 6disseminated ,07
PR4VIS4NA+ 2IAGN4SIS
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232
• 0ronchiectasis• ,ropical diseases 6dengue% malaria%
enteric fever% 3,I7
• Haemolytic anemia• HDH• ImmunodeAciency
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Investigations
• (0(;*nemia with mildthrombocytopenia
• H0;=.7 g3&l E0( 1$99!';4=G!D;1?G!;=G
• SR + 1/2hr;=9mm % 1hr ;119 hr8E+EVA,E2:
• 'latelet;(.07Dakhs/mm• 'eripheral smear microcytic
hypochromic anemia with mildthrombocytopenia no abnormal cells
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•
,I0(; F4$ mcg/dl! #(8;D-E! '(8;$• Sr .erritin; normal! #(H( ;normal• Hb electrophoresis;normal! Reticount
;2G• :(,;Negative
• R0S/R.,/D(,R-D<,S;N-R#*D
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• ,?-'(1@,?7'(10/ ,0ilirubin;
N-R#*D• ,rDA6B(ED! 5/:D!5D-;$• *D' ;1$
• (oagulation proAle normal• *05;respiratory alkalosis• Srammonia normal• (S. analysis ; normal
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• Man$oux
s$rongly6osi$i#e-))!!
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• 3rine R/;normal
•
0lood urine c/s;sterile• Stool (/S;No enteric growth
• 2engue Ig# lisa negative
•
+e6$o Ig#;negative• ,4RH proAle negative
• #';negative
•
Gas$ric Fuice AC1 6times7 ; negative
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, ,%orax-R$ !i&&le lo'e consoli&a$ion an& 13+no&ular o6aci$ies ;
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• ,reated with ampicillin K clo"acillin Kgentamicin
• No response % fever persisted• Suspected HDH
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• Sr.ibrinogen ;normal• ,riglyceride normal• 0one marrow aspirate normal• Diver biopsy to r/o I#; normal
• *ntibiotics changed to #eropenamand vancomycin
Fever %ersisted
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• .ungal Dung aspirate (/S Candida?rowth
• Started on Am%hotericin B ;1F days
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• (*, II *,, started
•
In$ensi#e 6%ase - )S0H0R00E0 ;(H0R00E0
• on$inua$ion 6%ase - 7H0R0E0
• Steroids added• Reasons for starting *,,
– .ever on B o since 2 months of life – 'ersistent pneumonia – .ailure to thrive
– #antou" strongly positive
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'RSIS,N, 'N3#-NI*
( < S , I (
. I 0 R - S I S OOOOOOOO
•(ystic Abrosis work up normal•Retroviral screening 6parents/child7 -#e•Immunoglobin proAle normal
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M.tuberculosis- catalase %ositive
organism - disorder of %hagocytosis
2B'
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HR4NIGRAN"+4MA,4"S
2ISEASE
:ue to Anancial constraint .low(ytometry not done
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2IS"SSI4N G2HR4NI GRAN"+4MA,4"S2ISEASE8G2:
In%eri$e& disorder of phagocyticactivity common in boyssusceptibility to recurren$ infec$ion
since 'ir$%#utations in NA2PH oxi&ase
subunits
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+INIA+MANICES,A,I4NS
Onset ; infancy to young adulthood• Recurrent pneumonia•
Hepatic or any other abcess• *nemia of chronic disease• 'oor growth• Hepatosplenomegaly• GRAN"+4MA formation
,I-D-5<;catalase
K8organisms ;
#tuberculosis!Serratia%
0urkholderia
%
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4NCIRMA,I4N• .low cytometry
6:HR7• N0, dye test
,REA,MEN,• HS(,• 'rophylactic
antibiotic Bantifungal
• (orticosteroids
• Interferon P