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Organic acidaemia crisis — RACP Paediatrics MCQ

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ModerateGeneticsOrganic acidaemia crisisRACP Paediatrics

A 2-year-old with propionic acidaemia presents with metabolic decompensation (acidosis, hyperammonaemia, ketosis) during gastroenteritis. What is the immediate management?

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Correct answer: B — Stop protein + IV dextrose

Organic acidaemia crisis: stop protein, high-rate IV dextrose (GIR 8-10 mg/kg/min) to suppress catabolism, IV carnitine, correct acidosis.

Reference: RCH Melbourne – 2023 – Metabolic Emergency CPG