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Congenital Hyperinsulinism
Background
Congenital Hyperinsulinism: Persistent Hyperinsulinaemic Hypoglycaemia of Infancy; PHHI; Nesidioblastosis (old name)
Congenital Hyperinsulinism (CH) is a rare inherited disorder in which the regulation of the secretion of insulin is faulty and there is an over production of insulin from the beta cell of the pancreas. Insulin is the most important hormone that controls the blood glucose (sugar) level. Too much insulin leads to hypoglycaemia (low blood glucose levels). If the hypoglycaemia is not treated it can cause brain damage, learning disability and even death. It is thought that CH occurs in about 1 in 40,000 live births with a male to female ratio of 1.3 to 1. Neonatal onset CH shows in the first days or weeks after birth and is the most severe form. Infant onset CH shows in the first few months, or even years, of life and is milder. A rare adult onset form of CH has been documented. CH occurs in all ethnic groups.
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