publication . Article . 2006

Molecular mechanisms of neonatal hyperinsulinism.

Irina Giurgea; Pascale de Lonlay; Maria Ribeiro; Claire Nihoul-Fékété; Francis Jaubert; Christine Sempoux; K Hussain; Jean-Jacques Robert; Francis Brunelle; Laurence Hubert; ...
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  • Published: 27 Sep 2006 Journal: Hormone Research in Paediatrics, volume 66, pages 289-296 (issn: 1663-2818, eissn: 1663-2826, Copyright policy)
  • Publisher: S. Karger AG
Abstract
Congenital hyperinsulinism (CHI), characterized by profound hypoglycaemia related to inappropriate insulin secretion, may be associated histologically with either diffuse insulin hypersecretion or focal adenomatous hyperplasia, which share a similar clinical presentation, but result from different molecular mechanisms. Whereas diffuse CHI is of autosomal recessive, or less frequently of autosomal dominant, inheritance, focal CHI is sporadic. The most common mechanism underlying CHI is dysfunction of the pancreatic ATP-sensitive potassium channel (K(+)(ATP)). The two subunits of the K(+)(ATP) channel are encoded by the sulfonylurea receptor gene (SUR1 or ABCC8) a...
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doi: 10.1159/000095938
Subjects
Medical Subject Headings: endocrine system
free text keywords: Pediatrics, Perinatology, and Child Health, Endocrinology, Diabetes and Metabolism, Endocrinology, Congenital hyperinsulinism, medicine.disease, medicine, Sotos syndrome, Perlman syndrome, medicine.medical_specialty, Dominance (genetics), Sulfonylurea receptor, ABCC8, biology.protein, biology, Hyperinsulinism, Glucokinase, Internal medicine
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