Gerba W M, Miller D R, Pahwa S, Cunningham-Rundles C, Gupta S
Am J Pediatr Hematol Oncol. 1982 Summer;4(2):155-60.
The clinical and laboratory features of a child with chronic granulomatous disease (CGD) and IgA deficiency and his family are presented. Bactericidal and NBT dye reduction studies confirmed the diagnosis of CGD in the patient and the carrier state in the mother. No other family member had IgA deficiency. The manifestations of the IgA deficiency include multiple autoimmune antibodies, progressive pulmonary dysfunction but no gastrointestinal or rheumatoid symptoms. The etiology of the IgA deficiency appears to be a failure in terminal B cell differentiation as evidenced by the presence of normal numbers of IgA bearing cells detected by a fluorescent monospecific antisera, a normal profile of T cell subpopulations, normal responses to the mitogens PHA, Con A, PWM, and antigens C. albicans, E. coli, and S. aureus, and the absence of suppressor cell activity in co-culture assays. The significance of the association of these two disorders is discussed.
本文介绍了一名患有慢性肉芽肿病（CGD）和IgA缺乏症的儿童及其家族的临床和实验室特征。杀菌和NBT染料还原研究证实了该患者患有CGD以及其母亲为携带者状态。没有其他家族成员患有IgA缺乏症。IgA缺乏症的表现包括多种自身免疫抗体、进行性肺功能障碍，但无胃肠道或类风湿症状。IgA缺乏症的病因似乎是终末B细胞分化失败，这一点可通过以下证据证明：用荧光单特异性抗血清检测到正常数量的携带IgA的细胞、T细胞亚群谱正常、对丝裂原PHA、Con A、PWM以及抗原白色念珠菌、大肠杆菌和金黄色葡萄球菌的正常反应，以及在共培养试验中不存在抑制细胞活性。讨论了这两种疾病关联的意义。
