USE OF SOMATOSTATIN IN NESIDIOBLASTOSIS

Description of two cases with severe neonatal hyperinsulinism due to nesidioblastosis in whom intravenous (i.v.). Somatostatin was used to improve their metabolic control. The first case is a newborn with persistent hypoglycemia despite the use of i.v. dextrose(20 mg/Kg/min), diazoxide(22 mg/Kg/day) and prednisone(1.5 mg/Kg/day). Somatostatin in a dosage or 8-10 ug/Kg/h our produced a marked increase in blood glucose concentration, allowed to withdraw the i.v. dextrose and to taper the dose of corticosteroids. Concomitantly the increasing volumes of oral feedings allowed a better nutritional state before subtotal pancreatectomy. The second case is a three months old girl with severe hyperinsulinism were Somatostatin was used, first before pancreatectomy at a dosage of 5 ug/Kg/hour with reduction of i.v. dextrose from 20 to 10 mg/Kg/min. After surgery the patient persisted with hypoglycemia and again Somatostatin allowed to mantain euglycemia when parenteral nutrition with increasing concentration of aminoacids was introduced. Finally after a second pancreatectomy and with persistent hypoglycemia Somatostatin in a dosage of 4 to 8 ug/Kg/hour produced a marked reduction of i.v. dextrose from 15 to 4 mg/Kg/min. In summary in accordance with previous reports, i.v. Somatostatin has proved to be useful in reducing the amount of i.v. dextrose, and in the management of hypoglycemia in patients with severe hyperinsulinism.