NEPHROTIC

SYNDROME
DR NAING NAING OO
SENIOTURER
 DEFINITION

• a clinical syndrome of massive proteinuria defined by

-- Proteinuria > 40 mg/m²/hour
-- Hypo-albuminaemia of < 25g/l
-- Oedema
• -- Hypercholesterolaemia
 CAUSES

• Primary or idiopathic: commonest type in children

• Secondary: HSP
SLE
Malaria, Hepatitis
Drugs – penicillamine, NSAID,
heavy metals
post-streptococcal glomerulonephritis
Malignancy: leukemia, lymphoma
 TYPES

Three main types

1. Minimal change: no changes seen under normal microscope
podocytes foot process fusion on electron microscope
2. Focal segmental glomerulosclerosis
3. Membranous nephropathy: Hepatitis B
Lymphoma
 PRESENTATION
• General features: lethargy and anorexia
• Hypoalbuminaemia: periorbital in the morning then other area later
Ascites
pleural effusion
• Proteinuria: frothy urine
• Hypo-gammagloulinaemia: risk of infection (especially encapsulated organisms –
streptoccus pneumonia
• Intravascular hypovolaemia: abdominal pain, circulatory collapse or venous
thrombosis
 INVESTIGATION

• FBC (normal Hb, WBC N/ neut leukocytosis if infection), PCV

• Renal profile: Urea, electrolytes and creatinine
• LFT: albumin (<25g/l)
• Serum cholesterol and triglycerides: both high
• ASO and Anti-DNAase B: (streptococcal infection)
• Hbs Ag for hepatitis
• Complement factor C3 and C4 (to exclude SLE and APSGN)
• ANF and double stranded DNA – for SLE
• Urine: dipstick for proteinuria (+3 or +4)
microscopy
albumin : creatinine ratio
• Throat swab: streptococcal infection
• Renal biopsy: not indicated because 80% of children with idiopathic
nephrotic syndrome have minimal change steroid responsive disease.
Indications of renal biopsy – protocol MCQ
• Indications for renal biopsy: steroid resistant nephrotic syndrome (failure
to achieve remission despite 4 weeks of adequate corticosteroid therapy)
atypical features at presentation: high
creatinine or < 1yearof age.
non-minimal change nephrotic syndrome –
persistent hypertension, renal impairment +/-
persistent haematuria
 COMPLICATIONS
• Hypovolemia
• Infection: (classically pneumococal) primary peritonitis (due to low
immunoglobulin)
• Intra-vasular thrombosis: renal vein thrombosis and DVT (due to
hypovolemia, hyper-coagulable state with a low anti-thrombin III
• Hyper-cholesterolemia.
• Acute tubular necrosis if severe hypovolemia.
 MANAGEMENT

• Daily weight, urine albumin and serum electrolytes and albumin and fluid
intake output chart
• A normal protein diet with adequate calories
• No added salt to the diet when child has oedema
• Penicillin V at diagnosis and during relapses
• Careful assessment of the haemodynamic status
 Hypovolaemia: Abdominal pain, cold peripheries, poor capillary refill,
poor pulse volume with or without low blood pressure.
Hypervolaemia: Basal lung crepitations, rhonchi, hepatomegaly
• Human albumin grossly oedematous states together with IV frusemide
 CORTICOSTEROID THERAPY
• Oral prednisolone 60 mg/m2/day x 4 week
and followed by
• Alternate day prednisolone 40 mg/m2/day x 4 week then
• taper off over 4 weeks then stop.
• 80% of children achieve remission within 28 days. (urine dipstix trace or
nil for 3 consecutive days)
 • Steroid resistant case: failure to achieve response to an initial 4 weeks
treatment with prednisolone at 60 mg/m²/day
• Relapse: urine albumin excretion > 40 mg/m²/hour or urine dipstix of ≥ 2+
Must know for 3 consecutive days.
• Frequent relapse: ≥ 2 relapses within 6 months of initial diagnosis or
≥ 4 relapses within any 12 month period
• Steroid dependent relapse: relapses occurring during steroid taper or
within 14 days of the cessation of steroids.
• Steroid sparing agent: cyclophosphamide
 REFERENCE

1. Illustrated Textbook of Paediatrics, 4th edition.

2. Nelson Textbook of Paediatrics, 11th edition.
3. Paediatric Protocols for Malaysian Hospitals, 4th edition.