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[家族性结节病。兄弟二人病例报告]

[Familial sarcoidosis. Report of 2 cases in brothers].

作者信息

Zielonka T M, Lasota A, Jaworski A, Szlapin M, Urbankowski A, Droszcz W

机构信息

Kliniki Pneumonologii Akademii Medycznej, Warszawie.

出版信息

Pneumonol Alergol Pol. 1996;64(5-6):340-4.

PMID:8924889
Abstract

For many years a genetic predisposition to sarcoidosis has been considered likely, however no convincing evidence have ever been found. We report histologically proven cases of sarcoidosis in two brothers. Initially, the case of sarcoidosis was diagnosed in a 36-year-old male patient (II stage of radiological lesions) and 4 years later the case of sarcoidosis was diagnosed in 28-year-old younger brother of the first patient (III stage). The brothers did not live together but, in the same neighbourhood. We evaluated the active evolution of a disease by clinical, radiological, including high resolution computed tomography and functional methods as well as by evaluating cellular and soluble components of bronchoalveolar lavage fluid (angiotensin converting enzyme, protein, phospholipids). The above constitutes the first report on familial sarcoidosis recorded in Poland.

摘要

多年来,结节病的遗传易感性一直被认为是可能的,但从未找到令人信服的证据。我们报告了两兄弟经组织学证实的结节病病例。最初,一名36岁男性患者(放射学病变II期)被诊断为结节病,4年后,第一名患者28岁的弟弟被诊断为结节病(III期)。兄弟俩不住在一起,但在同一街区。我们通过临床、放射学(包括高分辨率计算机断层扫描)和功能方法评估疾病的进展,同时评估支气管肺泡灌洗液的细胞和可溶性成分(血管紧张素转换酶、蛋白质、磷脂)。以上是波兰记录的首例家族性结节病报告。

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