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ADPKD — RACP Paediatrics MCQ

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ModerateNephrologyADPKDRACP Paediatrics

A 14-year-old presents with hypertension and a renal mass. His father and paternal grandmother had kidney surgery for cysts. Ultrasound shows bilateral renal cysts (3 cysts on one side, 2 on the other). There is no hepatic fibrosis. What is the most likely diagnosis?

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Correct answer: E — Autosomal dominant polycystic kidney disease

Bilateral renal cysts in an adolescent with a positive family history (autosomal dominant inheritance) and no hepatic fibrosis is ADPKD. Diagnostic criteria in children differ from adults; ≥3 total cysts in a child with a positive family history is significant. ADPKD (PKD1/PKD2 mutations) rarely causes ESKD in childhood but requires long-term monitoring. Tolvaptan is available for adults to slow cyst growth.

Reference: RACP Paediatric Curriculum – Nephrology/Genetics; RCH Melbourne – 2023