Premium
The pathogenesis of pneumothorax in B irt– H ogg– D ubé syndrome: A hypothesis
Author(s) -
Johannesma Paul Christiaan,
Houweling Arjan C.,
Waesberghe JanHein T.M.,
Moorselaar R.J. Jeroen A.,
Starink Theo M.,
Menko Fred H.,
Postmus Pieter E.
Publication year - 2014
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.12405
Subject(s) - medicine , pneumothorax , pathogenesis , lung , birt–hogg–dubé syndrome , cyst , pathology , surgery
The development and natural course of lung cysts in patients with B irt– H ogg– D ubé 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.