title: The pathogenesis of pneumothorax in Birt-Hogg-Dubé syndrome: a hypothesis.
abstract: The development and natural course of lung cysts in patients with Birt-Hogg-Dubé syndrome (BHD) is still unclear, and the relationship between lung cysts and pneumothorax is not fully clarified. Based on the follow-up results of thoracic imaging in six patients with BHD, we hypothesize that decreased potential for stretching of the cysts' wall and extensive contact with the visceral pleura are probably responsible for rupture of the cyst wall resulting in increased risk for pneumothorax.
authors.affiliation: Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Paul Christiaan
authors.initials: PC
authors.lastname: Johannesma
authors.forename: Arjan C
authors.initials: AC
authors.lastname: Houweling
authors.forename: Jan-Hein T M
authors.initials: JH
authors.lastname: van Waesberghe
authors.forename: R J Jeroen A
authors.initials: RJ
authors.lastname: van Moorselaar
authors.forename: Theo M
authors.initials: TM
authors.lastname: Starink
authors.forename: Fred H
authors.initials: FH
authors.lastname: Menko
authors.forename: Pieter E
authors.initials: PE
authors.lastname: Postmus
mesh_terms: D000328:Adult; D058249:Birt-Hogg-Dube Syndrome / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D016022:Case-Control Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted / Q000379:methods; D008168:Lung / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009426:Netherlands; D010994:Pleura / Q000473:pathology*; D011030:Pneumothorax / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D012008:Recurrence; D015203:Reproducibility of Results; D020775:Thoracic Surgery, Video-Assisted / Q000379:methods; D014057:Tomography, X-Ray Computed / Q000379:methods
keywords: Birt-Hogg-Dubé syndrome; folliculin; folliculin gene; pneumothorax; renal cell cancer