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Familial Persistent Hyperinsulinemic Hypoglycemia of Infancy and Mutations in the Sulfonylurea Receptor

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posted on 04.12.2015, 11:34 by M. J. Dunne, C. Kane, R. M. Shepherd, J. A. Sanchez, Roger F. L. James, Paul R. V. Johnson, A. Aynsley-Green, S. Lu, J. P. Clement, K. J. Lindley, S. Seino, L. Aguilar-Bryan, G. Gonzalez, P. J. Milla
[1st paragraph] Persistent hyperinsulinemic hypoglycemia of infancy is caused by inappropriate and excessive secretion of insulin. Although the disease is rare in outbred communities (approximately 1 case per 50,000 persons), the incidence is approximately 1 per 2500 in inbred Arabic communities in which there is a familial (autosomal recessive) form of the disease. The disease most commonly presents with severe hypoglycemia a few hours after birth, although some cases present after several weeks or months. Some patients have a response to treatment with diazoxide or somatostatin, but others require partial pancreatectomy to control the hyperinsulinism.

History

Citation

New England Journal of Medicine 1997; 336:703-706

Version

AM (Accepted Manuscript)

Published in

New England Journal of Medicine 1997; 336:703-706

Publisher

Massachusetts Medical Society

issn

0028-4793

eissn

1533-4406

Available date

04/12/2015

Publisher version

http://www.nejm.org/doi/full/10.1056/NEJM199703063361005

Language

en

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