Mendes E, Toledo S P
Allergol Immunopathol (Madr). 1975 Mar-Apr;3(2):95-8.
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.
对3名患有胡尔勒综合征(黏多糖贮积症Ⅰ型)的兄弟进行了细胞介导免疫研究。两名患者对感染性抗原、PHA刺激的反应以及对二硝基氯苯(DNCB)的致敏情况被认为是正常的。最年长的兄弟(16岁)在18天后出现DNCB致敏,而第二个兄弟(5岁)仅在25天后出现。最年幼的兄弟,2岁,对PHA刺激反应正常,仅对念珠菌素显示出微弱的皮肤反应,且未获得DNCB致敏。文中讨论了胡尔勒综合征患者在生命最初几年免疫机制存在延迟和部分缺陷的可能性。