OTC – sodium benzoate — RACP Paediatrics MCQ

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HardGeneticsOTC – sodium benzoateRACP Paediatrics

A 3-day-old neonate: encephalopathy, seizures, ammonia 1200, respiratory alkalosis, elevated glutamine, elevated urinary orotic acid. What specific treatment?

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Correct answer: B — IV sodium benzoate

Hyperammonaemia with respiratory alkalosis + elevated orotic acid = urea cycle defect (likely OTC). IV sodium benzoate (nitrogen scavenger). Dialysis if ammonia >500.

Reference: RCH Melbourne – 2023 – Metabolic Emergency CPG