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Archives of Disease in Childhood - Fetal and Neonatal Edition 2000;82:F87-F97; doi:10.1136/fn.82.2.F87
Copyright © 2000 BMJ Publishing Group Ltd & Royal College of Paediatrics and Child Health.
Arch Dis Child Fetal Neonatal Ed 2000;82:F87-F97 ( March )

Article

Hyperinsulinism of infancy: towards an understanding of unregulated insulin release Ruth M Shepherd, Karen E Cosgrove, Rachel E O'Brien, Philippa D Barnes, Carina Ämmälä, Mark J Dunne, on behalf of the EU funded European Network for Research into Hyperinsulinism in Infancy (ENRHI)

Institute of Molecular Physiology and Department of Biomedical Science, Sheffield University, Western Bank, Sheffield S10 2TN, UK

Correspondence to: Professor Dunne email: m.j.dunne{at}sheffield.ac.uk

Insulin is synthesised, stored, and secreted from pancreatic beta  cells. These are located within the islets of Langerhans, which are distributed throughout the pancreas. Less than 2% of the total pancreas is devoted to an endocrine function. When the mechanisms that control insulin release are compromised, potentially lethal diseases such as diabetes and neonatal hypoglycaemia are manifest. This article reviews the physiology of insulin release and illustrates how defects in these processes will result in the pathophysiology of hyperinsulinism of infancy.


Keywords: hypoglycaemia; ATP sensitive potassium channels; insulin secretion; nesidioblastosis; hyperinsulinism; diazoxide


© 2000 by Archives of Disease in Childhood

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