The polyglandular deficiency syndrome: a new variant in Persian Jews.
Five Persian Jews were detected with the polyglandular deficiency syndrome (PDS). Primary hypoparathyroidism and hypogonadism were present in each, adrenal insufficiency in two, and insulin-dependent diabetes mellitus and latent hypothyroidism in single subjects. The percentage of T and B cells, and the mononuclear cell response to phytohemagglutinin and Concanavalin A were normal in all five. IgG and IgA levels and the OKT4+/OKT8+ cell ratio were low in one subject. Antinuclear and antithyroid antibodies were present in one subject. HLA-DR5 was present in 4/4, HLA-24 and B5 (B51) in 3/4 subjects. A single case of isolated hypoparathyroidism (IHP) was detected among 12 first degree relatives. HLA antigens B8, DR3, were absent in all of these subjects. Seven non-Iranian Jews with IHP were also examined. HLA A26 or A25 were present in all seven. Persian Jews appear to have a unique variant of PDS.
D000293:Adolescent; D000309:Adrenal Insufficiency / Q000150:complications* / Q000276:immunology; D000328:Adult; D000974:Antibodies, Antinuclear / Q000032:analysis; D001402:B-Lymphocytes / Q000032:analysis / Q000187:drug effects; D003208:Concanavalin A / Q000494:pharmacology; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000276:immunology; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D007006:Hypogonadism / Q000150:complications* / Q000276:immunology; D007011:Hypoparathyroidism / Q000150:complications* / Q000276:immunology; D007037:Hypothyroidism / Q000150:complications* / Q000276:immunology; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007492:Iran / Q000208:ethnology; D007585:Jews; D008213:Lymphocyte Activation / Q000187:drug effects; D008297:Male; D009000:Monocytes / Q000032:analysis / Q000187:drug effects; D010835:Phytohemagglutinins / Q000494:pharmacology; D013577:Syndrome; D013601:T-Lymphocytes / Q000032:analysis / Q000187:drug effects
D000974:Antibodies, Antinuclear; D006680:HLA Antigens; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D010835:Phytohemagglutinins; D003208:Concanavalin A