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Steroid-resistant nephrotic syndrome — RACP Paediatrics MCQ

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HardNephrologySteroid-resistant nephrotic syndromeRACP Paediatrics

A 5-year-old with a typical first nephrotic presentation still has nephrotic-range proteinuria after 4 weeks of correctly dosed daily prednisolone. Adherence is confirmed and no secondary infection or malignancy is evident. What should occur before empiric calcineurin-inhibitor treatment?

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Correct answer: B — Arrange nephrology-led genetic testing and kidney-biopsy assessment before calcineurin inhibition

Explanation lettering: C = shown as A · E = shown as B · D = shown as C · A = shown as D · B = shown as E

E is correct. Failure to remit after four weeks requires paediatric-nephrology review. KDIGO 2025 encourages genetic testing in primary steroid-resistant nephrotic syndrome and indicates biopsy at four to six weeks, because a monogenic diagnosis or histology changes the value and risk of immunosuppression. A prolongs ineffective high-dose steroid exposure. B is not universal first-line treatment for steroid resistance. C bypasses diagnosis and essential safety assessment. D misclassifies a child who has never become steroid sensitive; relapse terminology requires prior remission. Calcineurin inhibition may follow in selected non-genetic primary disease, but it should not precede this diagnostic branch.

Reference: Royal Children’s Hospital Melbourne: Nephrotic syndrome: https://www.rch.org.au/clinicalguide/guideline_index/nephrotic_syndrome/ KDIGO 2025: Nephrotic syndrome in children: https://kdigo.org/wp-content/uploads/2025/04/KDIGO-2025-Guideline-for-Nephrotic-Syndrome-in-Children.pdf