[Clinical aspects, diagnosis and therapy of nesidioblastosis]

Hyperinsulinemia due to an excessive secretion of insulin independent on normal regulation is the most frequent cause of persistent neonatal hypoglycemia. We report on clinical course, diagnostic procedures and treatment of nesidioblastosis in three patients. Main symptoms observed in newborn period were hypoglycemia, respiratory embarrassment, cyanosis and convulsions. Primary treatment was started by continuous infusion of glucose, administration of diazoxide and prednisolone or glucagon. Most important investigations were performed simultaneously. In all three children subtotal resection of pancreas was necessary, because there was no constant blood glucose level. Histological specimens confirmed diagnosis. In two of three patients pancreatectomy followed. One suffers from diabetes mellitus, the other one fed normally, has stable blood glucose level possibly due to existence of extrapancreatic insulin producing cells.