PT - JOURNAL ARTICLE AU - Ruth M Shepherd AU - Karen E Cosgrove AU - Rachel E O'Brien AU - Philippa D Barnes AU - Carina Ämmälä AU - Mark J Dunne TI - Hyperinsulinism of infancy: towards an understanding of unregulated insulin release AID - 10.1136/fn.82.2.F87 DP - 2000 Mar 01 TA - Archives of Disease in Childhood - Fetal and Neonatal Edition PG - F87--F97 VI - 82 IP - 2 4099 - http://fn.bmj.com/content/82/2/F87.short 4100 - http://fn.bmj.com/content/82/2/F87.full SO - Arch Dis Child Fetal Neonatal Ed2000 Mar 01; 82 AB - 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.