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Related to Congenital hyperinsulinism: Congenital hypothyroidism


presence in the system of an above-normal amount of insulininsulin,
hormone secreted by the β cells of the islets of Langerhans, specific groups of cells in the pancreas. Insufficiency of insulin in the body results in diabetes. Insulin was one of the first products to be manufactured using genetic engineering.
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, the substance secreted by the pancreaspancreas
, glandular organ that secretes digestive enzymes and hormones. In humans, the pancreas is a yellowish organ about 7 in. (17.8 cm) long and 1.5 in. (3.8 cm) wide. It lies beneath the stomach and is connected to the small intestine at the duodenum (see digestive system).
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 and needed by the body to utilize sugar. An increased amount of insulin in the body results in below-normal amounts of sugar in the system, giving rise to such symptoms as headache, dizziness, weakness, and emotional instability. In severe cases there may be convulsions, coma, and death. The cause of oversecretion of insulin may be organic, i.e., a tumor of the pancreas, impaired liver function, or endocrine disorders, or it may be functional, e.g., unusual muscular exertion, pregnancy, or lactation. In diabetics, hyperinsulinism is known as insulin shock and may occur from overdosage with insulin in the course of treatment. Where there is some organic cause for hyperinsulinism, surgery may be required to eliminate it. Functional hyperinsulinism and insulin shock are treated by dietary measures designed to bring the insulin-sugar ratio into better balance.


Condition caused by abnormally high levels of insulin in the blood.
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Dysregulation of insulin secretion in children with congenital hyperinsulinism due to sulfonylurea receptor mutations.
Preoperative evaluation of infants with focal or diffuse congenital hyperinsulinism by intravenous acute insulin response tests and selective pancreatic arterial calcium stimulation.
The Congenital Hyperinsulinism Center at The Children's Hospital of Philadelphia is the only facility of its kind in the country, and one of the few centers worldwide, with the knowledge and capability to successfully cure patients.
Recently, he was the senior author of a study in developing a diagnostic test for newborns that identifies a rare, severe form of congenital hyperinsulinism that can be cured with organ-sparing surgery.

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