title: Clinical and immunologic features of selective IgA deficiency.
abstract: Selective absence of serum and secretory IgA is probably the most common form of human immunodeficiency. High frequencies of recurrent sinusitis, otitis media, pneumonia, and atopy were noted among a group of 75 such patients, all but 4 of whom were Caucasian. Seven instances of familial absence of IgA were detected among 106 relatives of 34 of the group; in 1 family 1 member from each of 3 successive generations was affected. Two IgA-deficient children were later found to have normal amounts of serum IgA. Despite their humoral deficit, B lymphocytes bearing surface IgA were detected in 9/9 IgA-deficient patients in immunofluorescence studies of their peripheral blood lymphocytes. Although in vitro lymphocyte responses to 2 putative T-cell mitogens and to allogenic cells were normal, results of spontaneous rosette formation studies with sheep erythrocytes raise the possibility of a lymphocyte subpopulation deficit in this condition.
mesh_terms: D000042:Absorption; D000818:Animals; D001260:Ataxia Telangiectasia / Q000209:etiology; D001327:Autoimmune Diseases / Q000209:etiology; D001402:B-Lymphocytes / Q000276:immunology; D001666:Binding Sites, Antibody; D002176:Candida albicans / Q000276:immunology; D004912:Erythrocytes / Q000276:immunology; D005260:Female; D005455:Fluorescent Antibody Technique; D006041:Goats / Q000276:immunology; D006801:Humans; D006968:Hypersensitivity, Delayed / Q000276:immunology; D017098:IgA Deficiency; D007104:Immune Adherence Reaction; D007106:Immune Sera; D005779:Immunodiffusion; D007072:Immunoglobulin D / Q000032:analysis; D007073:Immunoglobulin E / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000235:genetics; D008607:Intellectual Disability / Q000209:etiology; D008213:Lymphocyte Activation; D008297:Male; D010375:Pedigree; D011863:Radioimmunoassay; D011947:Receptors, Antigen, B-Cell; D012756:Sheep / Q000276:immunology; D012882:Skin Tests; D013045:Species Specificity
chemical_list: D007106:Immune Sera; D007072:Immunoglobulin D; D007075:Immunoglobulin M; D011947:Receptors, Antigen, B-Cell; D007073:Immunoglobulin E