Cell-mediated immunity in Hurler's syndrome.
Cell-mediated immunity was investigated in 3 brothers with Hurler's syndrome (Gargoylism). The response to infectious antigens and PHA stimulation and sensitization to DNCB was considered normal in two patients. DNCB sensitization occurred after 18 days in the oldest brother (16 years old) and only 25 days in the second brother (5 years old). The youngest brother, 2 years old, showed a normal response to PHA stimulation, a weak cutaneous reaction only to oidiomycin was found and the DNCB sensitization was not obtained. The possibility of a retarded and partial defect in the immune mechanism in patients with Hurler's syndrome during the first years of life is discussed.
D000293:Adolescent; D000935:Antifungal Agents; D002675:Child, Preschool; D004137:Dinitrochlorobenzene / Q000276:immunology; D006801:Humans; D006968:Hypersensitivity, Delayed / Q000276:immunology; D007111:Immunity, Cellular; D037102:Lectins; D008213:Lymphocyte Activation; D008297:Male; D008059:Mucopolysaccharidosis I / Q000276:immunology*; D012882:Skin Tests; D014248:Trichophytin; D014374:Tuberculin Test
D000935:Antifungal Agents; D004137:Dinitrochlorobenzene; D037102:Lectins; D014248:Trichophytin