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HNF1A-MODY — SCE Endocrinology MCQ

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HardDiabetesHNF1A-MODYSCE Endocrinology

A 17-year-old man has diabetes diagnosed after osmotic symptoms. His BMI is 19 kg/m², plasma glucose is 18.6 mmol/L and venous pH is 7.37. His mother has diabetes diagnosed at age 24 and responds to gliclazide. He has glycosuria at relatively low plasma glucose levels. What is the underlying pathophysiology?

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Correct answer: D — Reduced hepatocyte nuclear factor transcription affecting beta-cell function

The autosomal dominant family history, young onset and sulfonylurea sensitivity point towards HNF1A-MODY, a transcription-factor beta-cell disorder. Glucokinase-MODY causes mild stable fasting hyperglycaemia rather than symptomatic hyperglycaemia. The renal glycosuria clue is a classic HNF1A feature.

Reference: Diabetes UK monogenic diabetes guidance