[Congenital nesidioblastosis. Successful treatment with total pancreatectomy]

A 6,410 g newborn baby suffered from severe hypoglycemia despite therapy with high doses of diazoxide and glucagon as well as intravenous application of glucose. There was no persistent response of blood glucose to continuous infusion of somatostatin. A 85% pancreatectomy was performed at the age of 6 weeks, after biochemical findings had indicated hyperinsulinism. As the hypoglycemia reappeared postoperatively, the child underwent total pancreatectomy. Now, at the age of 9 months, the baby's growth and development is normal under substitution therapy with Pankreon and depot-insulin 0.2 U/kg/day.