Cited 12 times since 2014 (1.3 per year) source: EuropePMC Respirology (Carlton, Vic.), Volume 19, Issue 8, 1 1 2014, Pages 1248-1250 The pathogenesis of pneumothorax in Birt-Hogg-Dubé syndrome: a hypothesis. Johannesma PC, Houweling AC, van Waesberghe JH, van Moorselaar RJ, Starink TM, Menko FH, Postmus PE

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.

Respirology. 2014 11;19(8):1248-1250