Pediatrics · Pediatric Nephrology (Nephrotic, Nephritic, UTI, Congenital)

An 8-year-old child with idiopathic nephrotic syndrome (minimal change disease) has been on prednisolone for 8 weeks with complete remission. He is now in his third relapse within 12 months and has been on steroids for 6 of the last 12 months. Which of the following best defines his condition and guides alternative therapy?

  • A Steroid-resistant nephrotic syndrome — kidney biopsy and calcineurin inhibitor
  • B Steroid-dependent nephrotic syndrome — first try MMF alone
  • C Frequently relapsing nephrotic syndrome — consider cyclophosphamide or levamisole
  • D Secondary nephrotic syndrome — screen for SLE
Correct answer: C. Frequently relapsing nephrotic syndrome — consider cyclophosphamide or levamisole

Explanation

Frequently relapsing nephrotic syndrome (FRNS) is defined as ≥2 relapses within 6 months of initial response or ≥4 relapses in any 12-month period. Steroid-dependent nephrotic syndrome (SDNS) is defined as relapse during steroid tapering or within 14 days of stopping. This child's description fits FRNS. Steroid-sparing agents including cyclophosphamide (24-week course), levamisole, MMF, or calcineurin inhibitors (cyclosporin, tacrolimus) are used. Steroid-resistant nephrotic syndrome is failure to achieve remission after 8 weeks of prednisolone at full dose, which is different from FRNS.

Reference: Ghai Essential Pediatrics, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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