Hyperinsulinism of infancy: towards an understanding of unregulated insulin release

RM Shepherd, KE Cosgrove, RE O'Brien… - Archives of Disease in …, 2000 - fn.bmj.com
RM Shepherd, KE Cosgrove, RE O'Brien, PD Barnes, C Ämmälä, MJ Dunne
Archives of Disease in Childhood-Fetal and Neonatal Edition, 2000fn.bmj.com
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 …
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.
fn.bmj.com