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[伴反复气胸的Birt-Hogg-Dubé综合征;一例报告]

[Birt-Hogg-Dubé syndrome with repeated pneumothorax; report of a case].

作者信息

Matsuoka Yuki, Adachi Yoshin, Metsugi Hiroyuki, Tokushima Takeshi, Adachi Yoshiki

机构信息

Department of Thoracic Surgery, Matsue Medical Center, Matsue, Japan.

出版信息

Kyobu Geka. 2014 Sep;67(10):946-9.

PMID:25201377
Abstract

A 36-year-old man had been treated by thoracoscopic bullectomy because of left pneumothorax at another hospital. 2 weeks post-surgery, he was transferred to our hospital because of recurrent left pneumothorax. Chest computed tomography (CT) revealed multiple, irregular-shaped pulmonary cysts of various sizes predominating in the apex of both lungs. Thoracoscopy showed diffuse multiple thinwalled small cysts on the visceral pleura. Small cyst with air leakage was sutured and other cysts were cauterized. He was diagnosed as having Birt-Hogg-Dubé (BHD) syndrome by DNA sequence analysis of his FLCN gene.

摘要

一名36岁男性因左侧气胸在另一家医院接受了胸腔镜下肺大疱切除术。术后2周,因左侧气胸复发转入我院。胸部计算机断层扫描(CT)显示两肺尖部有多个大小不一、形状不规则的肺囊肿。胸腔镜检查显示脏层胸膜上有弥漫性多个薄壁小囊肿。对有漏气的小囊肿进行了缝合,其他囊肿进行了烧灼处理。通过对其FLCN基因进行DNA序列分析,他被诊断为患有Birt-Hogg-Dubé(BHD)综合征。

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