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.
References in periodicals archive ?
Diabetes in a nonpancreatectomized child with nesidioblastosis. Diabetes Care 2004;27:626-627.
(13) performed angiographic anesthesia for a case of nesidioblastosis with insulinoma and reported that an infusion of propofol and remifentanil did not impair hemodynamic stability in their study.
Single case reports mediastinal teratomas with either increased exocrine pancreatic activity [27] or associated with aberrant islet differentiation or nesidioblastosis [28].
Perren et al., "Persistent hyperinsulinemic hypoglycemia in 15 adults with diffuse nesidioblastosis," The American Journal of Surgical Pathology, vol.
(8) Similar to multiple endocrine neoplasia type 1 (MEN1) syndrome, patients with VHL disease can be associated with precursor lesions including ductuloinsular complexes (nesidioblastosis), islet dysplasia (referring to normal-sized or slightly enlarged islets <0.5 mm that contain neuroendocrine cells showing loss of the normal spatial and quantitative arrangement of the normal [alpha], [beta], [delta], and PP cell types), pancreatic micro-adenomatosis (when islet dysplasia is between 0.5-5 mm, it is termed microadenoma-, multiple microadenomas are termed microadenomatosis), and peliosis of the nontumorous islets (17) (Figure 2, A and B).
A safety concern that has been gaining attention is the late development of post-prandial hypoglycemia and nesidioblastosis after gastric bypass.
HI was first described in 1954 by MacQuarrie (1) as "idiopathic hypoglycemia of infancy." HI has subsequently been referred to by many names, including leucine-sensitive hypoglycemia, islet dysregulation syndrome, persistent hyperinsulinemic hypoglycemia of infancy, and nesidioblastosis (2).
La nesidioblastosis es una complicacion metabolica que puede amenazar la vida, y aunque no se presenta frecuentemente, hay que sospecharla cuando hay neuroglicopenia.
When my small patient, in whom nesidioblastosis had been newly diagnosed,[3] was returned to my care, little was needed but to follow along with the suggested treatment of diazoxide.
The search terms were "hyperinsulinism" or "CHI" or "nesidioblastosis" or "hypoglycemia" or "persistent hyperinsulinemic hypoglycemia of infancy" or "hyperinsulinemic hypoglycemia of infancy" or "HI" or "hyperinsulinaemic" or "hyperinsulinemic" and "Turkey" or "Turkish".
(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.
Pancreatic growth and function following surgical treatment of nesidioblastosis in infancy.