Alport Syndrome — RACP Paediatrics MCQ

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HardNephrologyAlport SyndromeRACP Paediatrics

An 8-year-old has persistent microscopic haematuria. Father had renal failure at 35. Renal biopsy shows thin GBM with areas of splitting and lamellation. Diagnosis?

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Correct answer: A — Alport syndrome

GBM thinning WITH splitting/lamellation + family history of renal failure = Alport syndrome. Pure thin GBM disease has uniform thinning without splitting.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Haematuria