Ito M, Nakagawa A, Hirabayashi N, Asai J
Department of Pathology, Nagoya University Hospital, Japan.
Virchows Arch. 1997 Feb;430(2):131-7. doi: 10.1007/BF01008034.
Pulmonary disease was studied in four patients with ataxia-telangiectasia. Immunodeficiency was characterized by lymphopaenia, hypo-gammaglobulinaemia and decreased T-cell response to phytohaemagglutinin stimulation in mixed lymphocyte cultures. All four patients died from respiratory failure. Autopsy revealed that all four patients suffered from bronchiolitis obliterans in all lobes. Immunohistochemical examination demonstrated expression of MHC class II antigens on bronchiolar epithelium. Pulmonary infections in ataxia-telangiectasia patients included a case of mycoplasma pneumonia, one of cytomegalovirus pneumonia and one of Pseudomonas aeruginosa infection. The aetiology and immunological background of bronchiolitis obliterans are discussed. Bronchiolitis obliterans is a characteristic finding in ataxia-telangiectasia and may be due to the underlying immune deficit.
对4例共济失调-毛细血管扩张症患者的肺部疾病进行了研究。免疫缺陷的特征为淋巴细胞减少、低丙种球蛋白血症以及在混合淋巴细胞培养中T细胞对植物血凝素刺激的反应降低。所有4例患者均死于呼吸衰竭。尸检显示，所有4例患者各肺叶均患有闭塞性细支气管炎。免疫组织化学检查显示支气管上皮细胞上有MHC II类抗原表达。共济失调-毛细血管扩张症患者的肺部感染包括1例支原体肺炎、1例巨细胞病毒肺炎和1例铜绿假单胞菌感染。文中讨论了闭塞性细支气管炎的病因及免疫背景。闭塞性细支气管炎是共济失调-毛细血管扩张症的一个特征性表现，可能归因于潜在的免疫缺陷。
