These pages are best viewed with Netscape version 3.0 or higher or Internet Explorer version 3.0 or higher. When viewed with other browsers, some characters or attributes may not be rendered correctly. Prompt diagnosis and treatment are required because of the high risk of permanent brain damage from the extremely low blood glucose.
Treatment often requires partial removal of the pancreas.
Until recently, the basis for this relentless inappropriate release of insulin by pancreatic -cells, in spite of low blood glucose, was unknown. HI is inherited as an autosomal recessive trait. Through the collaboration of physician-scientists from Hadassah Hospital in Israel and from Children's Hospital of Philadelphia, DNA from members of 15 families with two or more affected children was collected. The role of mutations in the SUR as a cause of HI can now be defined in various racial and ethnic groups. The results of these studies of a rare recessive disease have provided important new insights into the etiology of HI, as well as limited information for genetic counseling in selected families.