J P H Shield1. 1. Bristol Royal Hospital for Children, Department of Child Health, Bristol, UK. j.p.h.shield@bristol.ac.uk
Abstract
BACKGROUND: Nine distinct genetic conditions have been identified in the last 12 years causing neonatal diabetes mellitus through failure of normal pancreatic development, islet cell dysfunction or beta-cell destruction. This review will focus on the three conditions about which our understanding of the pathology - and in some cases the treatment options - has greatly increased: transient neonatal diabetes mellitus, permanent neonatal diabetes due to 'channelopathies' and immune dysregulation, polyendocrinopathy, enteropathy X-linked syndrome. CONCLUSIONS: Effective treatment of neonatal diabetes requires thorough understanding of the disease processes underlying this highly variable condition. As our knowledge of pancreatic development and physiology expands, so, too, do the treatment options for some patients. Copyright (c) 2007 S. Karger AG, Basel.
BACKGROUND: Nine distinct genetic conditions have been identified in the last 12 years causing neonatal diabetes mellitus through failure of normal pancreatic development, islet cell dysfunction or beta-cell destruction. This review will focus on the three conditions about which our understanding of the pathology - and in some cases the treatment options - has greatly increased: transient neonatal diabetes mellitus, permanent neonatal diabetes due to 'channelopathies' and immune dysregulation, polyendocrinopathy, enteropathy X-linked syndrome. CONCLUSIONS: Effective treatment of neonatal diabetes requires thorough understanding of the disease processes underlying this highly variable condition. As our knowledge of pancreatic development and physiology expands, so, too, do the treatment options for some patients. Copyright (c) 2007 S. Karger AG, Basel.
Authors: Alan P Kenny; Nancy A Crimmins; Deborah J G Mackay; Robert J Hopkin; Kevin E Bove; Mike A Leonis Journal: Pediatr Dev Pathol Date: 2009 Sep-Oct