Kongenitaler Hyperinsulinismus

Congenital hyperinsulinism is the most common cause of persistent hypoglycemia in infancy and childhood. This heterogeneous disease is caused by different genetic defects in the regulation of insulin secretion. Of clinical importance is to differentiate between focal and diffuse pancreatic disease. Focal disease can be treated by operative enucleation of the lesion. In diffuse disease conservative dietary and drug treatment is favored to establish euglycemia. While diazoxide is administered orally, the long-acting somatostatin analog octreotide is given subcutaneously. Diazoxide is the treatment of choice if this results in stable euglycemia. The response to diazoxide depends on the underlying genetic defect. The main aim of treatment is to prevent recurrent episodes of severe hypoglycemia.