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Original Article
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Volume 344:270-274 January 25, 2001 Number 4
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Malabsorption Due to Cholecystokinin Deficiency in a Patient with Autoimmune Polyglandular Syndrome Type I
Christoph Hogenauer, M.D., Richard L. Meyer, M.D., George J. Netto, M.D., Diana Bell, M.D., Katherine H. Little, M.D., Laura Ferries, M.D., Carol A. Santa Ana, B.S., Jack L. Porter, M.S., and John S. Fordtran, M.D.

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Autoimmune polyglandular syndrome type I is an autosomal recessive inherited disease caused by mutations in the autoimmune regulator gene.1 Its hallmarks are the failure of multiple endocrine glands due to an autoimmune process, susceptibility to chronic candida infection because of a T-cell defect, and dystrophy of ectodermal tissues.2 The most common endocrine manifestations are hypoparathyroidism and adrenal failure. Hypogonadism, hypothyroidism, type 1 diabetes mellitus, and hypopituitarism may also occur. Nonendocrine manifestations include enamel hypoplasia, nail dystrophy, keratoconjunctivitis, and pernicious anemia.2,3 Except for candidiasis, patients with this syndrome have no apparent susceptibility to infections.

About 20 percent of patients with autoimmune . . . [Full Text of this Article]

Case Report

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From the Departments of Internal Medicine (C.H., K.H.L., C.A.S.A., J.L.P., J.S.F.) and Pathology (R.L.M., G.J.N., D.B.), Baylor University Medical Center, Dallas; and the Deaconess Billings Clinic, Billings, Mont. (L.F.).

Address reprint requests to Dr. Fordtran at Baylor University Medical Center, 3500 Gaston Ave., Dallas, TX 75246.

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