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
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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