title: Impairment of cell-mediated immunity in ectodermal dysplasia with aplastic anemia.
abstract: A case of ectodermal dysplasia and aplastic anemia is presented in which a cell-mediated immunodeficiency led to a fatal Pneumocystis carinii infection. Elevated levels of IgG, IgA and IgD were present with normal specific antibody titres. A deficient cell-mediated immunity was documented by low T cell numbers, poor in vitro mitogenic responses, negative skin tests and by the histologic finding at autopsy of thymic dysplasia.
mesh_terms: D000741:Anemia, Aplastic / Q000150:complications / Q000276:immunology* / Q000473:pathology; D002452:Cell Count; D002648:Child; D004476:Ectodermal Dysplasia / Q000150:complications / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007111:Immunity, Cellular; D007070:Immunoglobulin A / Q000032:analysis; D007072:Immunoglobulin D / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D008297:Male; D008934:Mitogens / Q000494:pharmacology; D011020:Pneumonia, Pneumocystis / Q000209:etiology; D012882:Skin Tests; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D007070:Immunoglobulin A; D007072:Immunoglobulin D; D007074:Immunoglobulin G; D008934:Mitogens