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DI-058 Experience with diazoxide in congenital hyperinsulinism
  1. J Mateo Carmona,
  2. L Menéndez Naranjo,
  3. M Almanchel Rivadeneyra,
  4. M Muñoz Contreras,
  5. A Tomas Luiz,
  6. M Valderrey Pulido
  1. University Hospital Arrixaca, Pharmacy, Murcia, Spain


Background Diazoxide is indicated for the treatment of symptomatic hypoglycemia hyperinsulinism of diverse aetiology and diazoxide is used in congenital hyperinsulinism in neonates and infants.

Purpose To describe the experience of diazoxide in the treatment of congenital hyperinsulinism in tertiary hospital.

Material and methods We describe the case of two infants with persistent hypoglycemia due to hyperinsulinism. The following data were collected: age, sex, blood glucose, pre-treatment, dose and duration of treatment, and side effects.

Results In both cases the starting dose was 10 mg/kg/day divided in three doses.

Case 1: Infant 9 month old with blood glucose of 30 mg/dL with a diagnosis of focal congenital hyperinsulinism by pancreatic hyperplasia. He received dextrose infusions maintaining persistent hypoglycemia in 40–55 mg/dL. Diazoxide had answer within 24 h with blood glucose above 65 mg/dL. Two years later, the patient presented hypertrichosis in back and arms, which did not reach result in suspension of diazoxide, but required dose adjustment to 5 mg/kg/day in three divided doses, with good glycemic control.

Case 2: Infant 6 months old who presented blood glucose of 37 mg/dL with a diagnosis of diffuse congenital hyperinsulinism. He received dextrose infusions and enteral nutrition with a high content carbohydrate with no increase in blood glucose. The treatment was initiated with diazoxide, but was necessary to increase the dose to 15 mg/kg/day for blood glucose levels above 65 mg/dL. Several days later, he presented low urine output and increased blood pressure, so the physician was decided to initiate treatment with hydrochlorothiazide 1 mg/kg/day. One year later, he presented a petechial rash and the treatment was discontinued. Finally, the treatment with diazoxide is reintroduced without presenting new episode of thrombocytopenia and with good glycemic control.

Conclusion In both cases the use of diazoxide was effective in controlling blood glucose levels in persistent hypoglycemia.

References and/or acknowledgements No conflict of interest.

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