Endocrine Abstracts

Congenital hyperinsulinism (CHI) is characterised by unregulated insulin secretion from pancreatic β-cells. The most severe forms are associated with defects in SUR1 and Kir6.2 (encoded by ABCC8 and KCNJ11), which form KATP channels in β-cells. Diazoxide therapy often fails in the treatment of CHI and may be due to reduced cell surface expression of KATP channels. We investigated methods to increase surface expression of KATP<...

Congenital Hyperinsulinism (CHI) is primarily a β-cell disorder with an incomplete pathogenesis. The purpose of this study was to generate in vitro models of the disease for the purposes of investigating the relationship between gene defect and β-cell development and function. We obtained post-operative resections of pancreatic tissue from four patients with hyperinsulinism. The tissue was collagenase treated and maintained in cell culture condit...