一般社団法人日本呼吸器学会 公式サイト
日本呼吸器学会英文誌 Respiratory Investigation
日本呼吸器学会誌 増刊号 学術講演会プログラム 抄録集 検索用
日本呼吸器学会誌 増刊号 学術講演会プログラム 抄録集 全文PDF

Abstract

Full Text of PDF Full Text of PDF (390k)
Article in Japanese

Case Report

Pleural covering effectively prevents recurrent pneumothoraxes in patients with Birt-Hogg-Dubé syndrome

Yuko Ishimarua  Aiko Masunagaa  Taiyo Komatsua  Takashi Marutsukab  Makoto Suzukic  Takuro Sakagamia 

aDepartment of Respiratory Medicine, Kumamoto University Hospital
bDepartment of Thoracic Surgery, Kumamoto Chuo Hospital
cDepartment of Thoracic Surgery, Kumamoto University Hospital

ABSTRACT

Patients with Birt-Hogg-Dubé (BHD) syndrome typically develop lung lesions in their 20s and have recurrent and refractory pneumothoraxes. The development of a pneumothorax interferes with the social life and quality of life of most patients. Herein, we report a series of four patients (three sisters and a cousin) in whom a family history of pneumothorax led to a diagnosis of BHD syndrome. In all of the patients, pleural covering was effective in preventing recurrent pneumothoraxes. Those patients who had had recurrence after thoracic drainage or bullectomy had no recurrence after pleural covering.
Our findings suggest that pleural covering might prevent the deterioration in quality of life associated with recurrent pneumothoraxes in patients with BHD syndrome.

KEYWORDS

Birt-Hogg-Dubé (BHD) syndrome  Cystic lung disease  Pneumothorax  Pleural covering 

Received 12 Oct 2022 / Accepted 30 Jan 2023

AJRS, 12(3): 164-169, 2023

Google Scholar