3. nephrotic syndrome
Post on 01-Jun-2015
4.367 Views
Preview:
TRANSCRIPT
MUHAMMAD ALI BIN ABDUL RAZAKWAN AHMAD SYAZANI BIN MOHAMEDNADIAH MOHD NASIR
NAME: SUFIAH MAATREGISTRATION NUMBER: SB 00302319D.O.B: 5th NOVEMBER 2009GENDER : GIRLAGE: 1YEAR 1 MONTH OLDETHNIC GROUP: CAMBODIANDATE AND TIME OF ADMISSION: 4th DISEMBER 2010DATE OF DISCHARGE: -WARD OF ADMISSION: WARD 8C, HSBINFORMANT: FATHERRELIABILITY: GOODADDRESS: KG KUBU GAJAH, SG BULOH.
Sufiah, a 1 year old Cambodian girl was a referred case from private clinic to Hospital Sg Buloh due to generalize swelling of the body especially around the eyes(periorbital) and abdomen for further management.
Previously well until 10 days prior to admission Started to had fever.
10 days prior to admission Fever: Father claim that the fever as low grade fever and it
is on and off. no episode of seizure or convulsion Her parents gave her Paracetamol syrup, fever
subside but reoccur afterward.
Swelling:
3 days before admission. Periorbital swelling could be seen by her parents. Started to cough on and off.
2 days before admission. Abdomen was swelling as well as the periorbital
area. Went to private clinic. Suspects that she had been bitten by insect that
cause allergic reaction and cause the swelling. The doctor gave her anti-allergic drug ,cough medication and paracetamol for her fever.
1 day before admission Swelling –worse
–more prominent –more generalize to the whole body.
Another private clinic. Examine her and also tests her urine. The
result shown that her urine had high level of protein, thus the doctor referred this case to Hospital Sg Buloh for further management.
At Hospital Sg. Buloh (Emergency Department),the doctor rechecked her urine sample and gave her
1. Prednisolone 25mg OD2. IV albumin 20% 5 ml/kg over 2 hours3. IV frusemide 1 mg/kg4. Syrup penicillin V 125mg BD
Then, she was admitted to the ward.
System Complaints
General No loss of appetite,no weight loss
Respiratory No shortness of breath
Cardiovascular No diaphoresis during feeding and no cyanosis.
Gastrointestinal No constipation, no diarrheoa and no vomiting
Hematologic No pallor, no bleeding, no bruises
Genitourinary Decrease amount of urine,dark colour
Ear, nose and throat No ear and nose discharge
Central nervous No loss of consciousness, no seizure and no abnormal movement.
MusculoskeletalNo muscle weakness.no gross deformity
Skin No rash
She had never been hospitalized before and no surgical history.
Nil
ALLERGY HX Nil
Her mother was healthy during pregnancy and was not on medication. The mother went to clinic regularly for follow up for her pregnancy.
She was born on 5th November 2009 at Damansara Damai Clinic, full-term and by normal spontaneous vaginal delivery. Her birth weight was 2.7 kg and she was crying at birth.
No admission to the NICU. No other complication such as fever
and neonate jaundice.
Exclusive breastfeeding: 4 month At 5 month she already been
introduced to formula milk and semi-solid food.
The diet continues until today.
Completed the immunization up to date.
No complication such as rash or fever.
Up to her chronological age.
Gross motor: Able to walk with one hand held.
Fine motor: Neat pincer gripSpeech: She can talk 2-3 words with
meaning.Sosial: Shy and casting, could waves
‘bye bye’
Sofia Maat is the youngest children over 2 siblings. Her sister is 4 years old and currently healthy. Both her parents are alive and well. No family
members that had same problem like her.
Sister,4, stays with aunt, healthy
Sufiah Maat, 1 year old with fever for 10 days and generalize swelling 3 days prior to admission.
Mother 25, healthy
Father 30, healthy
She was active and happy at home. She stays in Kg Kubu Gajah, Sg buloh in a one storey village house.
She lives with her father, mother, sibling and aunt with all basic amenities.
Her mother is a housewife while her father work at night market. Monthly income of the family is RM800.
The area of their house is not dengue prone area.
Due to her condition, she must stay in the hospital for further monitoring.
This is her first admission to the Hospital so she quite irritable
Her mother had to look after her in the hospital. Leave her sister at home to be taken care by her
aunt. Economic status:
worried about the cost of her treatment> monthly income only RM800> Cambodians-might need to pay more compare to Malaysian citizen.
General condition Sufiah was sitting comfortably on her mother’s
lap. -She was conscious, alert and responsive to
people. -Not in pain -°dysmorphism -Her face looks puffy and swollen -°abnormal movement seen -Nutritional and hydration status was good -branula attached on her left dorsum
Vital signs Temperature : 36°C Blood pressure : 92/52 mmHg Pulse : 118 beat per
minute, normal volume, normal rhythm
Respiratory rate : 34 breathe per minute
Oxygen saturation : 100% Impression : She is currently
stable.
Anthropometry Weight : 8.7 kg Length : 71.0 cm Head circumference: 46.1 cm Impression : She is in 50th centile in
all anthropometry measurement
when checked on centile chart.
Examination for Hydration status
°sunken eyes tongue and mucous membranes in the
oral cavity were moist °loss of skin turgor. Capillary refill time was less than 2
seconds Impression: Her hydration status was
good.
Examination of Face, Head & Neck, Limbs
Appearance : °dysmorphism, bilateral periorbital swelling, face puffiness
Hands : Both hands slightly swollen Pallor : °pallor Cyanosis : °cyanosis Oral cavity : Good oral hygiene, moist mucous
membrane, °ulcer, pink tongue Eyes : °pallor, °jaundice, °discharge, °sunken eyes ENT : °ear and nose discharge, °throat redness,
°redness on her tymphanic membrane Shape of head : Normal head shape Neck : °thyroid enlargement, °abnormal pulsation Hair : °hair loss Extremities : °cyanosis at nail bed, °finger clubbing for
upper and lower extremities, °palmar erythema, and capillary refill time is less than two seconds, °koilonychias, °muscle wasting.
Oedema : There is bilateral leg pitting oedema up to midshin.
Impression : There was generalized oedema
Examination of back
°spinal deformities such as scoliosis, lordosis and kyphosis
°no tenderness °sacral oedema Impression: No abnormality detected
Examination of lymph nodes
°palpable lymph nodes in cervical, occipital, axillary and inguinal areas
Impression: No abnormality detected
Developmental assessment
Gross motor : Sufiah can stand up and walks with support.
Fine motor : She can do a pincer grasp as she picks up toys.
Social : She can hold bottle herself. Language & hearing : Sufiah has started to
say simple words and response when she was called.
Impression : Her development is corresponding with her milestone.
Cardio-vascular system
On inspection, her chest moves symmetrically with respiration. There was no chest wall deformity, no scar, no dilated veins, no precordial bulge, no sign of respiratory distress and no visible pulsation noted.
On palpation, apex beat was felt at 4th intercostals space, mid-clavicular line. There was no left parasternal heaves and no thrills at left sternal edge, pulmonary area and aortic area.
On auscultation, normal 1st and 2nd heart sound
was heard. There was no additional heart sound or murmur.
Impression: No abnormal findings
Respiratory system
On inspection, the chest moves symmetrically with respiration on both sides. There was no suprasternal, intercostals and subcostal recession. There was no chest deformity and no scar seen. The chest was not hyperinflated.
On palpation, the trachea is centrally located and chest expansion was symmetrical on both sides. The apex beat was located at 4th intercostals space, mid-clavicular line. Normal vocal fremitus was noted
On percussion, both sides of her mid clavicular, mid axillary, and scapular line segments of lungs were resonance. There was normal liver and cardiac dullness.
On auscultation, the air entry was adequate on both sides of the lung. Normal vesicular breath sound was heard. There were no added sounds heard.
Impression: No abnormal findings
Abdominal examination
On inspection, her abdomen was symmetrically distended and moves with respiration. The umbilicus was centrally located and inverted. There was no abnormal scar, no dilated vein, no visible pulsation and peristalsis noted.
On light palpitation, her abdomen was soft and non tender. On deep palpation, there was no tenderness, no mass felt and no hepatospleenomegaly. Both her kidneys were not ballotable
On percussion, there was positive shifting dullness and
fluid thrills. On auscultation, normal bowel sound present with no
renal bruit. Impression: Sufiah’s abdomen was distended with
fluid.
Musculoskeletal system °muscle wasting or hypertrophy on
upper and lower limbs °no bony deformity °signs of inflammation normal movement of joint
Impression: No abnormal findings.
Nervous system
Higher function:
-Mental status: She was conscious and response to people. No abnormal behaviour.
-Speech: She can say simple words. Cranial nerves: cranial nerves were intact. Motor function: Muscle bulk and muscle tone was normal.
Muscle power for all extremities grading 5/5. Biceps, triceps, supinator, knee, and ankle reflexes were present. Plantar response was normal with negative Babinski’s sign. The abdominal reflex was also normal.
Sensory functions:
A) Sensory: Normal sensation to touch, pain, temperature, vibration and joint position sense.
B) Signs of meningeal irritation: No neck stiffness with negative Brudzinski’s sign and Kernig’s sign.
Diagram of Body: Back & Front
Periorbital swelling and face puffiness
Distended abdomen with positive shifting dullness and fluid thrills
Bilateral pitting oedema up to mid shin
SUMMARY
Sufiah, a 13 months Cambodian girl was referred to the hospital with complaints of generalized swelling especially at her periorbital area and abdomen which started 3 days prior to admission. Her urine appeared cloudy, dark in colour and little in amount. Physical examination revealed generalized oedema with positive shifting dullness and fluid thrills.
PROVISIONAL DIAGNOSISNephrotic syndrome based on: Presence of generalised oedema Cloudy urine Oligouria Fluid thrills Positive shifting dullness Toddler age Weight gain (8 kg- 8.6 kg)
DIFFERENTIAL DIAGNOSIS
Points to support
Points to against
Acute gromerulonephritis
-Generalized oedema-Dark urine-Oligouria-Fluid thrills-Positive shifting dullness
-Toddler age
Cardiac failure -Generalized oedema-Fluid thrills-Positive shifting dullness
-Dark urine-Oligouria-Hypertension-Clubbing-Crepitations
INVESTIGATION General Investigations
full blood count
Impression: Platelet and white blood cell count were elevated
Result Normal range Remarks
WBC 22.0 4.5-13.5 x 10*9/L Increase
Hb 12.4 11.5-14.5 g/dL Normal
Plt 880 150-4– x 10*3 uL Increase
Haematocrit
37.2 37-45% Normal
Renal profile
Impression: Low creatinine level
Result Normal range Remarks
Urea 3.6 1.7-6.4 mmol/L Normal
Sodium 134 135-150 mmol/L Normal
Potassium 4.6 3.5-5 mmol/L Normal
Chloride 98 98.0-107.0 mmol/L Normal
Creatinine 27.7 44-88 mmol/L Decrease
Liver function test
Impression: There was markedly increased in total protein. This might be due to albumin infusion.
Result Normal range
Remarks
Total protein
45.0 6.3 - 7.9 g/dL
Increased
Albumin 8.0 3.5 - 5.0 g/dL
Increased
Globulin 37.0 9 - 48 U/L Normal
Bilirubin 1.8 0.1 - 1.0 mg/dL
Increased
Alanine transaminase
19 7 - 55 U/L Normal
Alanine transferase
217 45 - 115 U/L
Increased
Specific Investigations Urine Full Examination Microscopy Elements
(UFEME)
-protein: 3+
-blood: 3+
-nitrite,leukocyte, ketone: negative
Urine Protein Creatinine Index
-no result can be obtained from the medical record
Urine Culture and Sensitivity
-blood stain urine
-heavy mixed growth
Antistreptolysin O titre- to exclude post streptococcus glomerulonephritis
Anti-nuclear factor to exclude SLE
Serum complement (C3 and C4) to exclude post infectious glomerulonephritis and SLE.
FINAL DIAGNOSISIdiopathic Nephrotic Syndrome
On admission :
MEDICATION
1. Syrup penicillin V 125mg BD
2. Tablet prednisolone 25mg OD
3. IV albumin 20% 5 ml/kg over 2 hours
4. IV frusemide 1 mg/kg OD
Monitor Nephrotic Chart : Daily weight, Blood Pressure, Urine protein, Fluid intake
Nephrotic Syndrome
Definition
A collection of signs associated with glomerular disorders characterized by:
1) massive proteinuria (>3.5 g/day),2) hypoalbuminemia (serum albumin
levels <2.5 g/dL)3) hyperlipidemia ( >250 mg/dL) 4) generalized edema
Classification Nephrotic
syndrome
Primary/ Idiopathic Secondary
•Minimal change disease•Membranous GN•Focal segmental glomerulosclerosis•Membranoproliferative GN•IgA nephropathy
•Diabetes Mellitus•Amyloidosis•Systemic lupus erythematosus•Ingestion of drugs (lithium, penicillamine,”street heroin”)•Infections (malaria, syphilis, hepatitis B, HIV)•Malignancy ( carcinoma, melanoma)•Miscellaneous (bee-sting allergy, hereditary nephritis)
Congenital
Present during the first 6 months life• Finnish type is an autosomal recessive disorder most common in Scandinavian and due to mutation in component protein in the glomerulus filtration slit.• Diffuse mesangial sclerosis which is a heterogenous group of abnormalities.
Inflammatory reaction
Derangement in capillary walls of glomeruli
Increase permeability to plasma protein
Proteinuria
Allows protein to escape from plasma into glomerular filtrate
Drop in plasma colloid osmotic pressure
Fluid escapes into tissues Edema
Pathophysiology of Nephrotic Syndrome
Periorbital edema (earliest sign)Scrotal or vulval, leg, ankle edemaWeight gainAbdominal pain (Ascites)Respiratory distress (Pleural effusion)MalaiseDiarrhea
Nelson Essential Paediatrics Illustrated Textbook of Paediatrics
CLINICAL CLINICAL MANIFESTATIONMANIFESTATION
1. Urine protein – on test strips2. FBC and ESR3. Renal profile – urea, electrolyte, creatinine4. Serum cholesterol5. LFT - albumin6. Complement level7. Antistreptolysin O titre and throat swab
Nelson Essential Paediatrics Paediatric Protocols
INVESTIGATIONS
Imaging Studies
UltrasoundPulsed doppler studiesVoid cysturethrogram (VCUG)IV pyelogramMRICT
Diagnostic Studies
First morning specimen :Urine protein-to-creatinine ratio (normal : <0.2) Serum C3 complement level
RENAL BIOPSY : only indicated if a child does not respond to the prednisolone therapy within 4 weeks.
MANAGEMENT
Bed rest Diet – adequate calories ,normal protein
diet with salt restriction Antibiotic – penicillin V BD during relapse Fluid status :
assess for hemodynamic status. ( underfilling or overfilling )
Diuretic therapy Human albumin 25% parenterally with IV
loop diuretic ( frusemide ) to produce diuresis
1. Cortocosteroid Therapy Effective in inducing remission of NS Remission – urine dipstick is trace or nil for 3
consecutive days Relapse – urine albumin excretion > 40 mg /m²/hr OR
urine dipstix 2+ or > for 3 consecutive days Frequent Relapses
- Two or more relapses within 6 months of initial response or
- 4 or more relapses within any 12 month period
2. Cyclophosphamide therapy indicated for child who show signs of steroid toxicity
Initially-60 mg/m²/day for 4 weeks
Prednisolone 40 mg/m²/alternate day for 4 weeks then taper at 25% monthly over 4 month
1. Response
-Prednisolone 60 mg/m²/day till remission-40 mg/m²/alternate day for weeks then stop
2. RELAPSE
Reinduce (2), then taper & keep low dose alternate day prednisolone 0.1-0.5 mg/kg/dose for 6 month
3. Frequent relapse
Treat as (3) if not steroid toxic, consider cyclophosphamide if steroid toxic
4. Relapse while on prednisolone
2-3 mg/kg/day for 8-12 weeks (cumulative dose 168 mg/kg
5. Oral cyclophosphamide
-not steroid toxic: treat as 2 & 3- If steroid toxic paeds nephro
6. Relapse post cyclophosphamide
No response
Renal biopsy
1. Infection- bacteremia and peritonitis – patient with relapse have high risk of infection with capsulated bacteria esp pneumococus / E.coli
2. Side effect of steroid
3. Hypovolemia – result from use of diueresis or diarrhea
4. Hypercoagulable state with risk of tromboembolism – d/t loss of protein -> urinary lose of antithombin, thrombocytosis (steroid therapy) -> increase synthesis of clotting factor and increased blood viscosity ->predispose to thrombosis
THANK YOU
MINIMAL CHANGES DISEASES. Relatively benign disorder.
Most frequent cause of NS in children(1-7 years).
Clinical features Insidious development of NS. No hypertension and preserved renal
function. Good prognosis.
CAUSES
NEPHRITIC SYNDROMENephritic syndrome is defined by:
hematuria (usually with dysmorphic RBCs), and hypertension, oliguria (400 mL/day of urine). Uremia - due to retention of waste products Azotemia (elevated blood nitrogen)
Or come with symptom of underlying problems Triad of sinusitis, pulmonary infiltrates, and nephritis
suggesting Wegener granulomatosis Nausea/vomiting, abdominal pain, and purpura observed
with Henoch-Schönlein purpura Arthralgias associated with systemic lupus erythematosus
(SLE) Hemoptysis occurring with Goodpasture syndrome or
idiopathic progressive glomerulonephritis Skin rashes observed with a hypersensitivity vasculitis or
systemic lupus erythematosus; also possibly due to the purpura that can occur in hypersensitivity vasculitis, cryoglobulinemia, and Henoch-Schönlein purpura
Causes of Nephritic Syndrome
Post streptococal AGN Post infectious AGN Henoch schonlein perpura IgA nephropathy SLE Systemic vasculitis
Focal Segmental Glomerulosclerosis Lesion characterized histologically by
sclerosis. Pathogenesis is unknown. Clinical course: Little tendency for spontaneous remission
of idiopathic FSGS. Poor respond to steroid therapy. Bad prognosis.
CAUSES
Management Nephrotic Nephritic Normal protein dietNo added salt when
edemaPenicillin V at diag. &
during relapse esp. with gross edema
Diuretics is not necessary when steroid responsive
Human albumin-in grossly edematous
Hemodynamic status - Check for sign of hypervolamia or hypovolaemia
Strict monitoring-luid intake, urine output, daily weight, BP chart
Fluid restriction during oligouric phase
Diuretics Look for complication of
post strep AGN – hypertensive encephalopathy (usu. seizure), pulm edema (lft.vent failure), acute renal failure
Nephrotic vs nephritic
• Nephrotic syndrome:• 1. Massive proteinuria
2. Hypoalbuminemia3. Edema4. Hyperlipidemia/hyperlipiduria
• Nephritic syndrome:• 1. Hematuria
2. Oliguria3. Azotemia4. Hypertension
Side effects of steroids:1. Increased body weight2. Muscle wasting3. Growth retardation in
children4. Cutaneous striae5. Hypertension6. Increased susceptibility
to infections7. Delayed wound healing8. Hirsutism 9. Osteoporosis10. Diabetes peptic ulcer11. cataract
top related