Treatment for paediatric nephrotic syndrome,in flow chart

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Paediatric Nephrotic Syndrome - Treatment Algorithm

Here is the treatment flowchart from Comprehensive Clinical Nephrology, 7th Edition (Fig. 18.3):
Suggested Treatment Algorithm in Children With MCD

Flowchart Summary - Step by Step

STEP 1 - Initial Presentation

Child with biopsy-proven or suspected nephrotic syndrome (MCD)
  • Low-sodium diet, monitor for edema
  • IV albumin if diuretic-resistant, severely hypoalbuminaemic
  • Anticoagulation if albumin < 2 g/dL

STEP 2 - First-Line: Corticosteroid Therapy

Prednisolone/Prednisone:
  • Dose: 2 mg/kg/day (max 60 mg/day) OR 60 mg/m²/day
  • Duration: 4 weeks daily, then taper to 1.5 mg/kg/day (40 mg/m²/day) for 4 weeks on alternate days
  • ~95% of MCD children achieve complete remission within 4 weeks

STEP 3 - Assess Response at 4 Weeks


BRANCH A - Responder (~95%)

Complete remission achieved → stratify by relapse pattern:
PatternFrequencyNext Step
No relapses~25%No further therapy needed
Occasional relapses (IRNS)~25%Repeat course of corticosteroids; if no toxicity, continue; if continuing relapses → steroid-sparing therapy
Frequent relapses (FRNS)~50%See below
Steroid-dependent (SDNS)subsetSee below
Frequent Relapsers (FRNS):
  • First-line steroid-sparing: CNI (cyclosporin/tacrolimus) or cyclophosphamide
  • If remission achieved but still relapsing: Rituximab or MMF or levamisole (after remission induced)
  • Still relapsing after all above → Symptomatic therapy or repeat biopsy
Steroid-Dependent (SDNS):
  • First-line: CNI (calcineurin inhibitor) or rituximab
  • If CNI dependency/toxicity: → Rituximab or MMF or levamisole (after remission)
  • Still relapsing → Symptomatic therapy

BRANCH B - Nonresponder/SRNS (~5%)

No complete remission at 4 weeks = Steroid-Resistant NS (SRNS)
  • Renal biopsy mandatory
  • First-line: Calcineurin inhibitor (CNI) - cyclosporin or tacrolimus (± low-dose prednisolone)
    • CNI-responsive SRNS: partial remission at 6 months, complete remission at 12 months
    • If remission achieved but CNI dependency/toxicity → Symptomatic therapy + consider repeat biopsy
  • CNI-resistant SRNS: No remission → Symptomatic therapy + consider repeat biopsy

Key Definitions (ISKDC/KDIGO)

TermDefinition
SSNSComplete remission after 4 weeks of standard prednisolone
SRNSNo complete remission after 4 weeks of standard prednisolone
FRNS≥2 relapses/6 months or ≥4 relapses/12 months
SDNSRelapse during steroid taper or within 15 days of stopping
IRNS1 relapse/6 months or <4 relapses/12 months

General Supportive Measures (All Patients)

  • Diet: Low-sodium, normal protein
  • Oedema: Furosemide (cautiously - avoid hypovolaemia); IV albumin if severe
  • Thrombosis prophylaxis: LMWH or warfarin if albumin < 2 g/dL
  • Infection: Pneumococcal vaccination; penicillin prophylaxis during immunosuppression
  • Hyperlipidaemia: Statins (more relevant in prolonged or adult disease)

Sources:
  • Comprehensive Clinical Nephrology, 7th Ed., Chapter 18 (Fig. 18.3, Table 18.2)
  • Brenner and Rector's The Kidney, Chapter on Treatment of Nephrotic Syndrome
CNI = Calcineurin inhibitor (cyclosporin or tacrolimus) | MMF = Mycophenolate mofetil
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