Annals of The Japanese Respiratory Society:Pleural covering effectively prevents recurrent pneumothoraxes in patients with Birt-Hogg-Dubé syndrome

Vol.12 No.3 Table of Contents

Abstract

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Article in Japanese

Case Report

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

Yuko Ishimaru^a Aiko Masunaga^a Taiyo Komatsu^a Takashi Marutsuka^b Makoto Suzuki^c Takuro Sakagami^a

^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

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