Congenital Hyperinsulinism — RACP Paediatrics MCQ

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HardEndocrinologyCongenital HyperinsulinismRACP Paediatrics

A neonate born at 35 weeks has persistent hypoglycaemia (blood glucose <2.6 mmol/L) requiring IV dextrose infusion rates >10 mg/kg/min to maintain normoglycaemia. Insulin level is inappropriately elevated during hypoglycaemia. What is the first-line medical treatment?

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Correct answer: B — Diazoxide

Congenital hyperinsulinism: diazoxide (KATP channel opener) is first-line medical treatment. It suppresses insulin secretion. Diazoxide-unresponsive CHI (often due to ABCC8/KCNJ11 mutations) may require octreotide or surgical pancreatectomy. Prompt diagnosis and treatment prevent hypoglycaemic brain injury.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Persistent Neonatal Hypoglycaemia