PEDIATRICS

1. NEPHROTIC
SYNDROME
BY DR BRIGHT RAICE SIAMUNYANGA
12/30/2018BRIGHT RAICE SIAMS

2. OBJECTIVES
At the end of this lecture, students should
be able to
 Define Nephrotic syndrome
 Outline the etiologic
 List the Clinical manifestations
 List the Complications
 Outline the Management
12/30/2018BRIGHT RAICE SIAMS

3. DEFINITION
It is a kidey disorder that causes the your body to excrete
too much protein in urine.
This is a clinical syndrome charecterised by:
1. Heavy proteinuria >40 mg/m2/hr
2. Hypoalbuminemia-↓ serum albumin < 25 g/L
3. Oedema
4. Hypercholestrolnemia
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4. ETIOLOGY
 Primary or Idiopathic
 Secondary
 Congenital Nephrotic Syndrome
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5. Idiopathic / primary
(glomerular disease- intrinsic to kidneys)
 Minimal Change disease ( >80 % )
 Focal segmental Glomerulosclerosis (10%)
 Mesangial proliferation (5%)
 Membranous Nephropathy
 Membranoproliferative glomerulonephritis
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6. Secondary causes
 Infectious
Hepatitis (B,C) , HIV-1, Malaria (plasmodium malariae), Syphilis,
Toxoplasmosis
Post-infectious GN, HIV
 Systemic Disease
SLE, HSP (Henoch- Schonlein purpura) , DM
 Toxins and allergen
Bee sting, Food allergens
 Neoplastic
Lymphoma, Leukemia
 Drugs
Penicillamine, Gold, NSAIDS, Mercury, Lithium12/30/20
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7. Congenital NS
 Appears in the first 3 months of life
 Most cases are due to genetic causes
 Mutations in the gene encoding nephrin and those encoding
podocin
 NS in the first 3 months of life may also be part of multisystemic
syndromes such
 Pierson Syndrome -(congenital nephrotic syndrome and distinct
ocular abnormalities like microcoria (small pupils )
 Nail-patella Syndrome-Nail-patella syndrome is an autosomal
dominant disorder characterized by dysplasia of finger nails,
skeletal anomalies, and, frequently, renal disease.
 Denys- Dash syndrome -(triad of congenital nephropathy, Wilms
tumor, and intersex disorders)
 Congenital infections such as syphilis and CMV
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8. CLINICAL MANIFESTATION
 Sudden onset of pitting edema
- periorbital
- scrotal or vulva
- ankle or leg
 Weight gain
 Ascites
- abdominal pain
- malaise
 Diarrhea (due to intestinal edema)
 Respiratory distress (due to pulm. edema)
 Decreased urine output
 ±HTN and Hematuria
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9. COMPLICATIONS
1) Thromboembolic disorders
2) infections
3) Acute Kidney Failure
4) Pumonary Edema
5) Hypothyroidism
6) Hypocalcaemia
7) Anemia
8) PEM
9) Hypercholestremia
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10. Lab Investigations
 Urine Examination
 Full Blood Count and differential count
 Renal parameters :
 Spot Urine Protein : Creatinine ratio
 Urinary protein excretion
 Liver Function Tests ( eg. serum albumin)
 Renal Function Tests
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11. • Urinalysis - 3+ to 4+ proteinuria
• Renal Function
 Spot UPC ratio > 2.0
 UPE > 40 mg/m2/hr
 Serum Creatinine – normal or elevated
 Serum albumin - < 25 g/L
 Serum Cholesterol/ TGA levels – elevated
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12.  Other investigations
 complement levels: C3, C4
 Antistreptolysin O titre and throat swab
 Hepatitis B antigen
 Hepatitis C
 RPR
 Renal Biopsy
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13. Indications for Renal Biopsy
 Age below 12 months
 Gross or persistent microscopic hematuria
 Low blood C3
 Hypertension
 Impaired renal Function
 Failure of steroid therapy
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BRIGHT RAICE SIAMS

14. DEFINITION FOR DX & TX OF IDIOPATHIC NS
REMISSION:
 Urinary protein excretion < 4 mg/m2/hour or urine dipstix nil/trace for
3 consecutive days.
RELAPSE:
 Urinary protein excretion > 40 mg/m2/hour or urine dipstix ++ or more
for 3 consecutive days after treatment ceasation
INFREQUENT RELAPSE
 3 or less relapses per year
FREQUENT RELAPSES:
 Two or more relapses within 6 months of initial response or four or
more relapses within any 12 month period.
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15. STEROID DEPENDENCE:
 Two consecutive relapses occurring during the period of steroid
taper or within 14 days of its cessation.
STEROID SENTITIVE:
 Normalization of proteinuria within 4 weeks after start of standard
initial therapy with daily oral prednisolone
STEROID RESISTANCE:
 Failure to achieve remission in spite of 4 weeks of standard
prednisolone therapy.
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16. Initial Episode
NON MEDICAL
 Dietary advice; High protein diet, Salt moderation.
 Abulation
 Education
MEDICAL
 Corticosteroid therapy with Prednisolone
 ( 2mg/kg per day for 6 weeks followed by
1.5 mg/kg single morning dose on alternate days for
6 weeks )
OR
 60 mg/m2/day (max 80mg/day) for 6 weeks
 40 mg/m2/48 hr (max 60mg/dose) for further 6 weeks
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 BRIGHT RAICE
 Treatment of infections
 If significant edema – diuretics can
be used but cautiously SIAMS

17. • Symptomatic therapy:
 diuretic
 blood pressure control : ACEi (captopril, enalapril), angiotensin II
receptor antagonist
 hyperlipidaemia
• Immunosuppressive therapy:
Steroids
cyclophosphamide
Cyclosporin, tacrolimus, mycophenolate mofetil
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18. SIDE EFFECTS OF PREDINISIOLONE
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19. DIFFERENTIAL DIAGNOSIS OF
NS
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20. SUMMARY
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21. 12/30/2018BRIGHT RAICE SIAMS