Congenital Hyperinsulinism (CHI) is a rare genetic disorder of pancreatic β-cells that secrete excess amount of insulin causing severe persistent hypoglycemia (low blood glucose) in new-born babies and children.
Insulin, a hormone secreted by the β-cells of the islets of Langerhans of pancreas, is chiefly involved in the regulation of carbohydrate (glucose) metabolism. Any increase in blood glucose (sugar) concentration is the most important physiologic factor leading to insulin secretion. Insulin in turn leads to break down of glucose (glycolysis) into energy thereby decreasing glucose concentration in the blood. Additionally, insulin also prevents formation of new glucose (gluconeogenesis) conversion of fat into ketones (ketogenesis), which act alternative fuel for the body in absence of glucose.
