Nesidioblastosis associated with hyperinsulinemic hypoglycemia in adults: review of the literature.

Nesidioblastosis is a term that was first introduced by Laidlaw in 1938 to define the diffuse proliferation of pancreatic islet cells budding from ductal epithelium. First described in neonates, it is widely recognized to be the primary cause of persistent hyperinsulinemic hypoglycemia in infants. In adults, insulinoma accounts for most cases of hyperinsulinemic hypoglycemia. Adult-onset nesidioblastosis associated with hyperinsulinemic hypoglycemia is a very rare entity, representing 0.5-5% of cases of organic hyperinsulinemia. Since 1981, when Harness et al. reported the first series of adult nesidioblastosis causing hypoglycemia, only 71 cases have been published. This article provides a guide for the diagnosis of organic hyperinsulinism and indicates when nesidioblastosis should be suspected. New diagnostic methods and treatment options for this rare disease are proposed. We also present a review of all reported cases of adult nesidioblastosis in the last 22 years.