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Previous Volume 336:703-706 March 6, 1997 Number 10 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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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.^1,2,3

It has recently been suggested^2,3 that mutations . . . [Full Text of this Article]

Case Report

Methods

Genetic Analysis

Construction of SUR1 and Kir6.2 Expression Plasmids

Cell Culture and Transfection

Recombinant Expression System

Results

Clinical Findings

Genetic Analysis

Functional Studies of Isolated Beta Cells

Functional Studies of Reconstituted K_atp Channels

Discussion

Source Information

From the Department of Biomedical Science, University of Sheffield, Sheffield (M.J.D., C.K., R.M.S.); the Department of Surgery, University of Leicester, Leicester (R.F.L.J., P.R.V.J.); and the Institute of Child Health, University of London, London (A.A.-G., K.J.L.) — all in the United Kingdom; the Research Centre for Pathogenic Fungi and Microbial Toxicosis (J.A.S.) and the Division of Molecular Medicine (S.S.), Centre for Biomedical Science, Chiba University School of Medicine, Chiba, Japan; and the Departments of Cell Biology and Medicine, Baylor College of Medicine, Houston (S.L., J.P.C., L.A.-B.). Other authors were Gabriela Gonzalez, B.S., and Peter J. Milla, M.D.

Address reprint requests to Dr. Aguilar-Bryan at the Division of Endocrinology, Department of Medicine, Baylor College of Medicine, 1 Baylor Plaza, Rm. 537E, Houston, TX 77030.

References

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