An inverted ratio for T-helper/T-suppressor cells, and selective deficiency of cell-mediated immunity, in a girl with cartilage hair hypoplasia.
Clinical and immunological data are presented in a thirteen-year-old girl with cartilage-hair hypoplasia. From early infancy, the girl suffered from recurrent respiratory infections, and at ten years of age she had a severe and protracted attack of varicella. Immunologic investigations revealed very low mitogenic responses to PHA and ConA and an inverted ratio for T-helper/T-suppressor cells. However, antibody-mediated immunity was normal. The results emphasize that selective cell-mediated immune dysfunction, which is most often found in short-limbed dwarfism with cartilage-hair hypoplasia, may be due to increased T-suppressor- as well as decreased T-helper cell activity.
D000293:Adolescent; D002356:Cartilage / Q000002:abnormalities; D002644:Chickenpox / Q000150:complications; D004392:Dwarfism / Q000276:immunology*; D005260:Female; D006197:Hair / Q000002:abnormalities*; D006801:Humans; D007111:Immunity, Cellular; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D012141:Respiratory Tract Infections / Q000150:complications; D013577:Syndrome; D013601:T-Lymphocytes; D050378:T-Lymphocytes, Regulatory