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Congenital Hyperinsulinism

  • N. Scott Adzick
Chapter

Abstract

Congenital hyperinsulinism (HI) is a rare derangement of glucose metabolism that occurs with an estimated incidence of 1–1.4 in 50,000 live births, accounting for about 80–120 new cases in the United States each year. Higher rates of 1 in 2,500 live births have been reported in areas of high consanguinity, such as the Arabian Peninsula. Inappropriate over secretion of insulin is the hallmark of HI. The old term “nesidioblastosis” should be discarded. HI is the most common cause of persistent hypoglycemia in neonates and can lead to seizures and irreversible brain damage. Pancreatectomy for management of persistent infantile hypoglycemia was first performed at The Children’s Hospital of Philadelphia (CHOP) in 1950.

Keywords

Common Bile Duct Pancreatic Head KATP Channel Focal Lesion Normal Pancreas 
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Suggested Reading

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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of SurgeryChildren’s Hospital of Philadelphia, University of Pennsylvania School of MedicinePhiladelphiaUSA

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