Endogenous hyperinsulinism: diagnosis, management, and long-term follow-up

Persistent hyperinsulinemic hypoglycemia of the neonate (PHHN) usually presents in the neonate or infant. Although rare, the possibility of PHHN should be considered in all neonates who have unremitting hypoglycemia. Untreated hypoglycemia results in severe neurological disability or death. An inappropriately elevated serum insulin level and low serum ketone and free-fatty-acid concentrations in the presence of hypoglycemia confirm the diagnosis. Early diagnosis and aggressive medical management have reduced the morbidity associated with PHHN. Pancreatic resection is necessary for long-term control of hypoglycemia when medical therapy fails. Ninety-five percent pancreatectomy has been the procedure of choice at the author's institution and other pancreatic centers. However, a review of the authors' experience and of in the English-language literature has demonstrated a 33% failure rate, requiring further surgery or medical management. Furthermore, long-term follow-up showed that diabetes develops in most children who undergo 95% pancreatectomy. The high failure rate of 95% pancreatectomy and the ultimate development of diabetes in virtually all children suggest the alternative approaches should be considered.