Hyperinsulinemic hypoglycemia due to adult nesidioblastosis in insulin-dependent diabetes

Abstract

In neonates, persistent hyperinsulinemic hypoglycemia (PHH) is associated with nesidioblastosis. In adults, PHH is usually caused by solitary benign insulinomas. We report on an adult patient who suffered from insulin-dependent diabetes mellitus, and subsequently developed PHH caused by diffuse nesidioblastosis. Mutations of the MEN1 and Mody (2/3) genes were ruled out. Preoperative diagnostic procedures, the histopathological criteria and the surgical treatment options of adult nesidioblastosis are discussed. So far only one similar case of adult nesidioblastosis subsequent to diabetes mellitus II has been reported in the literature. In case of conversion of diabetes into hyperinsulinemic hypoglycemia syndrome, nesidioblastosis in addition to insulinoma should be considered.

Occult sporadic insulinoma: Localization and surgical strategy

“…Hypoglycemia due to excessive endogenous insulin secretion can be caused by a primary pancreatic beta cell disorder including an insulinoma, beta cell hyperplasia or nesidioblastosis (presence of diffuse microadenomatosis, in which multiple small non-encapsulated tumors or nodules are distributed throughout the pancreas), a beta cell secretagogue including a sulphonylurea or a beta cellstimulating antibody, or an antibody to insulin [20,[26][27][28][29] . The differential diagnosis of hypoglycaemia includes hormonal deficiencies, hepatic insufficiency, exogenous hyperinsulinism, medication, drugs and enzyme defects.…”

Nesidioblastosis and Pancreatic Non-functioning Islet Cell Tumor in an Adult with Type 2 Diabetes Mellitus

“… 9 In fact, there are reported cases of nesidioblastosis associated with pre-existing diabetes in adults. 8 , 10 We speculate that islet hyperplasia and nesidioblastosis in diabetic patients could be a reactive process, reflecting a compensating mechanism for β cell destruction or functional insufficiency.…”

An unusual cause of hypoglycemia in a middle-aged female after bariatric surgery

“…NIPHS is a syndrome characterized by hypoglycemia caused by increased insulin secretion from pancreatic beta cell hyperplasia. The diagnosis is based on exclusion of insulinoma and dumping syndrome by laboratory evaluation and histopathological examination of the pancreas ( 1 ). In our patient, we were able to exclude insulinoma due to the absence of fasting hypoglycemia with normal insulin, proinsulin, C-peptide levels, and negative imaging studies of the pancreas ( 2 ).…”