The pathogenesis of pneumothorax in Birt-Hogg-Dubé syndrome: A hypothesis

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 decrease...

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Veröffentlicht in:	Respirology (Carlton, Vic.) Vic.), 2014-11, Vol.19 (8), p.1248-1250
Hauptverfasser:	Johannesma, Paul Christiaan, Houweling, Arjan C., van Waesberghe, Jan-Hein T.M., van Moorselaar, R.J. Jeroen A., Starink, Theo M., Menko, Fred H., Postmus, Pieter E.
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Birt-Hogg-Dube Syndrome - complications Birt-Hogg-Dube Syndrome - diagnosis Birt-Hogg-Dube Syndrome - physiopathology Birt-Hogg-Dubé syndrome Case-Control Studies Disease Progression Female folliculin folliculin gene Humans Image Processing, Computer-Assisted - methods Lung - pathology Male Middle Aged Netherlands Pleura - pathology pneumothorax Pneumothorax - etiology Pneumothorax - pathology Pneumothorax - physiopathology Recurrence renal cell cancer Reproducibility of Results Thoracic Surgery, Video-Assisted - methods Tomography, X-Ray Computed - methods
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Beschreibung
Zusammenfassung:	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.
ISSN:	1323-7799 1440-1843
DOI:	10.1111/resp.12405