Familial extensive idiopathic bilateral pleural fibrosis
Familial extensive idiopathic bilateral pleural fibrosis
复制标题
DOI：
10.1034/j.1399-3003.1999.14d41.x
复制
发表时间：
1999-10-01
影响因子：
24.3
通讯作者：
Tazi, A
中科院分区：
文献类型：
--
作者：
Azoulay, E;Paugam, B;Tazi, A
作者报告了三姐妹患有不明原因的双侧孤立性心尖胸膜纤维化，对经验性抗结核治疗和口服皮质类固醇没有反应。这种疾病以一种无情的方式演变，在肺基部产生胸膜纤维化，导致两姐妹死亡，另一姐妹接受肺移植。没有其他家族性疾病或近亲病史，概述了这些病例的具体特征以及与其他明显的隐源性胸膜纤维化报告的差异。
The authors report three sisters,vith bilateral isolated apical pleural fibrosis of unknown origin, which did not respond to empirical antituberculosis therapy and oral corticosteroids. The disease evolved in an unrelenting fashion producing pleural fibrosis at the lung bases and leading to the death of two sisters and to lung transplantation in the other one. There was no history of other familial disease or consanguinity, The particular features of these cases and the differences from other reports of apparently cryptogenic pleural fibrosis are outlined.