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Hyperinsulinism of infancy: towards an understanding of unregulated insulin release
  1. Ruth M Shepherd,
  2. Karen E Cosgrove,
  3. Rachel E O'Brien,
  4. Philippa D Barnes,
  5. Carina Ämmälä,
  6. Mark J Dunne on behalf of the EU funded European Network for Research into Hyperinsulinism in Infancy (ENRHI)
  1. Institute of Molecular Physiology and Department of Biomedical Science, Sheffield University, Western Bank, Sheffield S10 2TN, UK
  1. Professor Dunne email: m.j.dunne{at}


Insulin is synthesised, stored, and secreted from pancreatic β 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.

  • hypoglycaemia
  • ATP sensitive potassium channels
  • insulin secretion
  • nesidioblastosis
  • hyperinsulinism
  • diazoxide

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