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The Capital Region of Denmark - a part of Copenhagen University Hospital
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Birt-Hogg-Dubes syndrom er en sjældent, men vigtig årsag til pneumothorax

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Birt-Hogg-Dubé syndrome (BHD) is an autosomal-dominant genodermatosis manifesting as a multisystemic disorder. The most common presentation of pulmonary involvement is spontaneous pneumothorax, and it has recently been estimated, that up to 5-10% of patients with primary spontaneous pneumothorax have underlying BHD. The purpose of this case report is to stress the paramount importance of a thorough family history in patients with spontaneous pneumothorax and to recommend, that patients with a spontaneous pneumothorax and a personal or family history of pneumothorax should be screened for BHD.

Translated title of the contributionBirt-Hogg-Dubé syndrome is a rare but important cause of pneumothorax
Original languageDanish
JournalUgeskrift for Laeger
Volume180
Issue number5
ISSN0041-5782
Publication statusPublished - 29 Jan 2018

ID: 56202579