Two cases of persistent hyperinsulinemic hypoglycemia that showed spontaneous regression and maturation of the Langerhans islets

Near-total pancreatectomy has been recommended as the treatment for persist ent hyperinsulinemic hypoglycemia (PHH) in infants. However, recently there has been a report described that one third of 95% pancreatectomy failed to prevent hypoglycemia and more than two thirds had diabetes ultimately. The authors experienced 2 cases of PHH, which raise a query about the extensiv e pancreatectomy. Case 1: A female patient who manifested PHH shortly after birth underwent less extensive pancreatectomy twice at age 2 months and 8 years. After each operation, her clinical symptoms regressed, and she becam e free from the disease eventually. Histologic findings showed nesidioblast osis in which the islets clearly matured. Case 2: A male infant with PHH ha d an absolute indication for pancreatectomy. However, after a meticulous co ntrol of the blood glucose level with parenteral nutrition followed by cont inuous enteral nutrition feeding combined with medication, he became free f rom the disease. The current cases show there exist cases of PHH in which t he islets mature and symptoms regress spontaneously. Therefore, we conclude near-total pancreatectomy is not always the treatment of choice. As an alt ernate strategy, long-term controlled feeding and medication combined with or without less extensive pancreatectomy should be considered with the expe ctation of spontaneous regression. Copyright (C) 2000 by W.B. Saunders Comp any.