Partial pleural covering for intractable pneumothorax in patients with Birt-Hogg-Dube Syndrome

摘要

Background and AimsBirt-Hogg-Dube syndrome (BHD) is an inherited disorder associated with a germline mutation of the folliculin (FLCN) gene. Most patients with BHD have multiple pulmonary cysts, and are at high risk of repeated pneumothorax. Although an increasing number of patients are diagnosed with BHD by genetic testing, therapeutic approaches for intractable pneumothorax have not yet been described.