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The pathogenesis of pneumothorax in Birt-Hogg-Dubé syndrome: a hypothesis
Paul Christiaan Johannesma1, Arjan C Houweling, Jan-Hein T M van Waesberghe
1Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands.
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.
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