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KCNJ11 neonatal diabetes — SCE Endocrinology MCQ

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HardDiabetesKCNJ11 neonatal diabetesSCE Endocrinology

A 9-month-old boy has diabetes diagnosed at 6 weeks of age and remains on basal-bolus insulin. Genetic testing confirms a KCNJ11 activating mutation. He has developmental delay and intermittent hypoglycaemia with feeds. What is the most appropriate management?

Educational content. Not a substitute for clinical judgement or local policy.

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Correct answer: A — Transfer from insulin to high-dose sulfonylurea under specialist supervision

KCNJ11 neonatal diabetes often responds dramatically to sulfonylurea therapy, but transfer must be specialist-led because doses and monitoring differ from type 2 diabetes. Continuing insulin indefinitely misses a disease-modifying treatment. The clinical pearl is that neonatal diabetes is genetic until proved otherwise.

Reference: International Society for Paediatric and Adolescent Diabetes guidance, BNF