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Glucose intolerance and diabetes are observed in the long-term follow-up of non-pancreatectomized patients with persistent hyperinsulinemic hypoglycemia of infancy due to mutations in the ABCC8 gene

Pediatric Endocrine Service. Children's Hospital Vall d'Hebron, Autonomous University and CIBERER. Barcelona, Spain

ancarrascosa{at}vhebron.net

ABSTRACT

Objective: The long-term follow-up of 3 non-pancreatectomized patients with persistent hyperinsulinemic hypoglycemia of infancy (PHHI) due to mutations in the ABCC8 gene is reported.

Research design and Methods.: Oral glucose tolerance test (OGTT) and venous 24-hour glucose-insulin profile were performed yearly from adolescence.

Results.: Patient 1 (now aged 31) developed insulin-dependent diabetes at the age of 25. In patient 2 (now aged 17) impaired fasting glucose and a diabetic OGTT response with normal HbA1c values have been observed since the age of 10. In patient 3 (now aged 24) intolerant OGTT response and hyperglycemic episodes with normal HbA1c have been observed since the age of 16. All patients presented relatively low insulin levels during hyperglycemia, normal body mass index and negative autoantibodies (anti-GAD, -IA2 and –ICA).

Conclusion.: Development of glucose metabolism impairment ranging from glucose intolerance to insulin-dependent diabetes is observed in the evolution of these patients.

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