LAUSR

Congenital hyperinsulinism (CHI) is the most common cause of severe persistent hypoglycemia in infants with an estimated incidence of 1 in 50 000 live births.1 Treatment of CHI includes medical, surgical, or combined therapies: oral diazoxide is the first-line medication, often ineffective due to inactivating mutations in the genes encoding the ATP-sensitive potassium channel. Second-line agents include the use of parenterally administered somatostatin analogs such as octreotide. Newer options such as continuous subcutaneous octreotide infusion and long-acting somatostatin analogs may not be feasible and safe for the use during the first weeks of life; therefore, conventional therapy with multidose daily octreotide injections is often required with a considerable physical and emotional impact on the quality of life of newborns and their caregivers. Furthermore, parents are usually in constant fear of hypoglycemia, since irreversible brain damage and permanent developmental issues may result, with consequent constant monitoring of blood glucose.2 This report describes a diazoxide-unresponsive patient with a focal form of CHI due to a novel mutation in ABCC8 successfully treated with octreotide by a subcutaneous injection port and a continuous glucose monitoring system (CGMS) to simplify glycemic control.