nesidioblastosis


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ne·sid·i·o·blas·to·sis

(nē-sid'ē-ō-blas-tō'sis),
Hyperplasia of the cells of the islets of Langerhans.
[nesidioblast + G. -osis, tumor]

nesidioblastosis

(1) A term first used in 1938 by Laidlaw for the neodifferentiation of islets of Langerhans from pancreatic ductal epithelium, which is clinically characterised by severe recurrent hypoglycemia and inappropriate elevation of serum insulin, C-peptide, and proinsulin. It was later renamed persistent hyperinsulinemic hypoglycemia of infancy (PHHI; some authors prefer congenital hyperinsulinism—CH). It is the most common cause of hyperinsulinism in neonates and, if untreated, can lead to hypoglycaemic brain damage or death. Once regarded as exclusive to infants and children, it may rarely occur in adults. PHHI/CH responds poorly if at all to medical management, mandating subtotal pancreatectomy.
(2) Acquired hyperinsulinism in adults caused by beta cell hyperplasia post-GI surgery.

nesidioblastosis

(nē-sīd″ē-ō-blăs-tō′sĭs)
Diffuse hyperplasia of the beta cells in the islets of Langerhans of the pancreas. It is a rare cause of hypoglycemia.

nesidioblastosis

pancreatic ductular and islet cell proliferation in children and in dogs with islet cell neoplasms.
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References in periodicals archive ?
19) The background pancreas characteristically lacks nesidioblastosis or hyperplasia of the islets of Langerhans, in contrast with neuroendocrine tumors associated with the multiple endocrine neoplasia syndromes.
A safety concern that has been gaining attention is the late development of post-prandial hypoglycemia and nesidioblastosis after gastric bypass.
HI has subsequently been referred to by many names, including leucine-sensitive hypoglycemia, islet dysregulation syndrome, persistent hyperinsulinemic hypoglycemia of infancy, and nesidioblastosis (2).
It is important to mention nesidioblastosis to dispel its continued association with HI.
beta] Cell nesidioblastosis in idiopathic hypoglycemia of infancy.
78) Recent data also suggests that patients may be at risk of hyperinsulinemic hypoglycemia due to nesidioblastosis after gastric bypass, perhaps due to trophic factor stimulation of beta cells, and all bariatric patients should be closely monitored for signs of hypoglycemia which may present in a similar manner as dumping syndrome.
Hyperinsulinemic hypoglycemia with nesidioblastosis after gastric-bypass surgery.
The triad of nesidioblastosis, congenital neuroblastoma and glomerulocystic disease of the newborn: a case report.
Meckel diverticulum is also described in association with inguinal (Littre hernia) and Spigelian hernias, intussusception, inversion resulting in acute and chronic gastro-intestinal blood loss, carcinoid and other rare tumors, and nesidioblastosis to name a few unusual affiliations.
Nesidioblastosis is a hyperfunctional disorder of pancreatic insulin-producing cells characterized by hypertrophic [beta] cells within enlarged or normal-appearing islets, small scattered endocrine cell clusters, and ductuloinsular complexes.
Coexistence of endocrine and exocrine tumors of the pancreas with nesidioblastosis is of particular interest because of the direct implication as to the origin and histogenesis of pancreatic endocrine cells and the relationship between endocrine and exocrine cells.
In addition, diffuse (multifocal) islet cell hyperplasia, ie, nesidioblastosis coexisting with a discrete islet cell tumor, is also uncommon.