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A Syndrome of Immunoglobulin A Deficiency, Diabetes Mellitus, Malabsorption, and a Common HLA Haplotype: Immunologic and Genetic Studies of Forty-Three Family Members

DAVID H. VAN THIEL, M.D.; WILLIAM I. SMITH Jr., M.D.; BRUCE S. RABIN, M.D., Ph.D.; STANLEY E. FISHER, M.D.; and ROGER LESTER, M.D.
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▸Requests for reprints should be addressed to David H. Van Thiel, M.D.; 1000G Scaife Hall, University of Pittsburgh School of Medicine; Pittsburgh, PA 15261.


Pittsburgh, Pennsylvania


Ann Intern Med. 1977;86(1):10-19. doi:10.7326/0003-4819-86-1-10
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Three persons in a kindred of 43 had variable expression of a syndrome consisting of immunoglobulin A deficiency, diabetes mellitus, malabsorption, and a common HLA haplotype. Findings from the proband included lifethreatening malabsorption; idiopathic intestinal mucosal atrophy with inflammation; IgA deficiency and antibodies to multiple endocrine organs; insulin-dependent diabetes mellitus; and the major histocompatibility antigens HLA-A2, B8, and DW3. In addition to the described syndrome other conditions present in the family include Graves' disease, vitiligo, hypocomplementemia, rheumatic fever, multiple sclerosis, and a nigh frequency of antibodies to endocrine tissue. Since Graves' disease, diabetes mellitus, and idiopathic Addison's disease have all been described in association with HLA-B8 and DW3, we believe that the occurrence of these diseases in this family suggests that a single immune response gene or gene complex is linked with HLA-B8 and DW3.

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