Nephrotic Syndrome
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Direct answer
Periorbital puffiness that parents mistake for allergy, progressing to dependent oedema with frothy urine in a preschool child, is nephrotic syndrome when the urine carries 3+ protein or more with serum albumin below 2.5 g per dL — and in children over 80 per cent of cases are minimal change disease, steroid-sensitive and biopsy-free. The first episode is treated with daily prednisolone at 2 mg per kg (60 mg per square metre) for four weeks, then alternate-day dosing with tapering per the IAP regimen; the drama afterwards is in the vocabulary — relapse, frequent relapse, steroid dependence, steroid resistance — which decides everything from levamisole to renal biopsy.
What you must remember
- The definition: proteinuria of 40 mg per square metre per hour or more (dipstick 3+ and above, or a protein-to-creatinine ratio above 2) with hypoalbuminaemia below 2.5 g per dL, oedema and hypercholesterolaemia.
- Minimal change disease dominates paediatric practice (over 80 per cent, unlike adults) and presents with normal blood pressure, renal function and complement — a quiet syndrome apart from the oedema.
- First-line steroid regimen: prednisolone 2 mg per kg per day or 60 mg per square metre (maximum 60 mg) daily for four weeks, then 1.5 mg per kg or 40 mg per square metre on alternate mornings for four to eight weeks with tapering; remission means urine protein negative or trace for three consecutive days.
- Relapse is 3+ proteinuria for three consecutive days; two or more relapses within six months (or four in a year) make frequent relapse; relapse on alternate-day steroid or within two weeks of stopping defines steroid dependence.
- Steroid resistance — no remission after eight weeks of adequate steroids — triggers renal biopsy and a shift to calcineurin inhibitors (tacrolimus or ciclosporin), with focal segmental glomerulosclerosis the usual histology behind it.
- Frequently relapsing and steroid-dependent children: levamisole 2 mg per kg on alternate days is the Indian and IAP favourite steroid-sparing agent, escalating to mycophenolate, cyclophosphamide or rituximab.
- Complications: spontaneous bacterial peritonitis (pneumococcus classically, and E. coli), cellulitis, thromboembolism from urinary loss of antithrombin III, hypovolaemia with abdominal pain and oliguria, and hypocalcaemia.
- Supportive care in relapse: no added salt, fluid restriction only while oedema is florid, a normal-protein diet, home dipstick monitoring, and pneumococcal with influenza vaccination.
- Biopsy at the outset only for: age under one year or beyond about eleven, hypertension, significant haematuria, low C3, azotaemia, or steroid resistance; contrast with post-streptococcal glomerulonephritis — haematuria with casts, hypertension and a low C3 weeks after a sore throat.
Eight weeks with a four-year-old
A four-year-old has two weeks of morning facial puffiness, now pitting oedema to the knees; urine 4+ protein, albumin 1.8 g per dL, cholesterol high, blood pressure and creatinine normal, microscopy bland. This is the steroid-sensitive picture of minimal change disease, and no biopsy is done. Prednisolone 2 mg per kg daily starts; by day four the dipstick reads trace, by day seven negative — remission. Daily therapy completes four weeks, alternate-day dosing follows with tapering, and the mother learns the dipstick and the vocabulary, because upper respiratory infections trigger most relapses. She is counselled on no added salt, daily dipsticks, and urgent return for fever with abdominal pain — peritonitis until excluded. The branches from here: a relapse is treated with daily steroid until remission then alternate-day; frequent relapses bring levamisole; failure to remit by eight weeks means steroid resistance, biopsy and tacrolimus.
Where students slip
The vocabulary is the commonest stumble: relapse, frequent relapse, steroid dependence and steroid resistance get used interchangeably when each has a definition and a different therapy. The second is dietary — prescribing a high-protein diet, or banning salt for life rather than during relapse. The third is fluid logic: oedema means hypoalbuminaemia, not volume overload, so aggressive diuretics in a hypovolaemic child can precipitate shock and thrombosis; albumin infusion is reserved for symptomatic hypovolaemia or massive oedema. And the contrast question — nephrotic versus nephritic — goes wrong whenever the examiner adds haematuria or hypertension to the vignette; those features move the child out of the quiet minimal-change box.
Frequently asked questions
What numbers define nephrotic syndrome in children?
Proteinuria of 40 mg per square metre per hour or more (dipstick 3+, or a protein-to-creatinine ratio above 2) with serum albumin below 2.5 g per dL, accompanied by oedema and hyperlipidaemia.
What is the first-line steroid regimen for the initial episode?
Prednisolone 2 mg per kg per day (60 mg per square metre, maximum 60 mg) for four weeks, then 1.5 mg per kg alternate mornings for four to eight weeks with tapering.
What defines steroid-resistant nephrotic syndrome?
Failure to remit after eight weeks of adequate steroid therapy — an indication for renal biopsy and calcineurin inhibitors.
Which drug is the preferred steroid-sparing agent in frequently relapsing disease in India?
Levamisole 2 mg per kg on alternate days, with mycophenolate, cyclophosphamide or rituximab as escalation.
When is a renal biopsy performed before treatment?
Age under one year or beyond about eleven, hypertension, significant haematuria, low C3, azotaemia, or steroid resistance — the steroid-responsive preschool child is spared.