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Organic acidaemia crisis — RACP Paediatrics MCQ

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ModerateGeneticsOrganic acidaemia crisisRACP Paediatrics

A 2-year-old with known propionic acidaemia presents with metabolic decompensation (metabolic acidosis, hyperammonaemia, ketosis) triggered by gastroenteritis. What is the most important initial management?

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Correct answer: E — Stop protein intake + IV dextrose

In organic acidaemias (propionic, methylmalonic, isovaleric acidaemia), metabolic crises are triggered by catabolic stress (illness, fasting, surgery). Immediate management includes stopping all protein intake, providing high-rate IV dextrose (10% initially, aiming for GIR 8-10 mg/kg/min) to suppress catabolism, IV carnitine, and correcting acidosis. Protein is reintroduced gradually once stable.

Reference: RCH Melbourne – 2023 – Metabolic Emergency CPG; RACP Paediatric Curriculum – Metabolic