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Mitchell E. Geffner, MD; Barbara M. Lippe, MD; Robert M. Itami, MD; Solomon A. Kaplan, MD; Baiba K. Gillard, PhD; Seymour R. Levin, MD; Ian L. Taylor, MD

Am J Dis Child. 1984;138(7):677-680.

Abstract
• An 18-year-old man had cystic fibrosis (CF) and insulin-resistant carbohydrate intolerance characterized by (1) obesity, basal hyperinsulinemia, and hyperglucagonemia; (2) impaired oral glucose tolerance; (3) hyperinsulinemia in response to oral and intravenous (IV) administration of glucose and to IV administration of tolbutamide; (4) exaggerated gastric inhibitory polypeptide secretion following orally administered glucose; and (5) diminished sensitivity to insulin administered IV compared with other patients with CF. Both parents also demonstrate basal and stimulated hyperinsulinemia in response to orally administered glucose. The long-term outlook for patients with CF is improving, and more patients are surviving childhood. Thus, it should be recognized that an insulin-resistant form of carbohydrate intolerance may develop in patients with CF with obesity and/or genetic risk factors.

(AJDC 1984;138:677-680)

Author Affiliations
From the Department of Pediatrics, UCLA Medical Center (Drs Geffner, Lippe, Itami, Kaplan, and Gillard), and the Departments of Medicine, Veterans Administration Wadsworth Medical Center (Dr Levin) and VA Sepulveda Medical Center (Dr Taylor), Los Angeles.

Footnotes
Read in part before the joint meetings of the Western Section, American Federation for Clinical Research, Western Society for Pediatric Research, and Western Region, the Society for Investigative Dermatology, Carmel, Calif, Feb 18, 1982, and at the annual meeting of the Society for Pediatric Research, Washington, DC, May 12, 1982.

Reprint requests to Department of Pediatrics, UCLA Medical Center, Los Angeles, CA 90024 (Dr Geffner).

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