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Division of Endocrinology, Metabolism, and Genetics, Department of Medicine, University of Kansas Kansas City, Kansas 66103
Division of Allergy, Immunology, and Rheumatology Department of Medicine, University of Kansas Kansas City, Kansas 66103
AAddress requests for reprints to: Dr. Richard S. Fairchild, Room 413 C, Division of Metabolism, Endocrinology, and Genetics, University of Kansas Medical Center, 39th and Rainbow, Kansas City, Kansas 66103.
Three brothers, two with Addison's disease and a monozygous twin discordant for adrenal insufficiency, presented an unique opportunity to evaluate the endocrine and immunological abnormalities associated with this disorder. None of the brothers had clinical evidence of other autoimmune disease. However, each of the twins had elevated titers of antithyroglobulin and antiparietal cell antibodies, and both Addisonian siblings had cytoplasmic islet cell antibodies. We evaluated the ability of nonspecific Concanavalin A-activated suppressor cells from all three siblings to inhibit immunoglobulin biosynthesis by pokeweed mitogen-stimulated B cells, cell proliferation by phytohemagglutinin-stimulated T cells, and the proliferative response of an allogeneic mixed lymphocyte culture. In comparison to normal controls, suppressor cells from the Addisonian siblings wereless efficient in inhibiting both B and T cell activities. The nonAddisonian twin had a lesser degree of impaired suppressor cell function to the B and T cell targets. Suppressorcell activity, as measured by the ability to inhibit proliferation within the mixed lymphocyte culture, was normal in all three siblings. The relationship of suppressor cell dysfunction, genetic predisposition, and the expression of the autoimmune state are discussed.
* This work was supported in part by Grant RR-828 from the General Clinical Research Centers Program of the Division of Research Resources, NIH Grant AI-15360, the V.A., the Oklahoma Chapter of the Lupus Foundation, and the Kansas Chapter of the Arthritis Foundation.
Received February 4, 1980.
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C. Betterle, C. Dal Pra, F. Mantero, and R. Zanchetta Autoimmune Adrenal Insufficiency and Autoimmune Polyendocrine Syndromes: Autoantibodies, Autoantigens, and Their Applicability in Diagnosis and Disease Prediction Endocr. Rev., June 1, 2002; 23(3): 327 - 364. [Abstract] [Full Text] [PDF] |
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