Literature DB >> 25302759

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

Paul Christiaan Johannesma1, Arjan C Houweling, Jan-Hein T M van Waesberghe, R J Jeroen A van Moorselaar, Theo M Starink, Fred H Menko, Pieter E Postmus.   

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.
© 2014 Asian Pacific Society of Respirology.

Entities:  

Keywords:  Birt-Hogg-Dubé syndrome; folliculin; folliculin gene; pneumothorax; renal cell cancer

Mesh：

Year:  2014        PMID： 25302759     DOI： 10.1111/resp.12405

Source DB:  PubMed          Journal:  Respirology        ISSN： 1323-7799            Impact factor:   6.424


  5 in total

1.  Loss of FLCN inhibits canonical WNT signaling via TFE3.

Authors:  John C Kennedy; Damir Khabibullin; Thomas Hougard; Julie Nijmeh; Wei Shi; Elizabeth P Henske
Journal:  Hum Mol Genet       Date:  2019-10-01       Impact factor: 6.150

Review 2.  Birt-Hogg-Dubé syndrome: a case report and a review of the literature.

Authors:  Dea Kejlberg Jensen; Anders Villumsen; Anne-Bine Skytte; Mia Gebauer Madsen; Mette Sommerlund; Elisabeth Bendstrup
Journal:  Eur Clin Respir J       Date:  2017-02-20

3.  Characterization of CT scans of patients with Birt-Hogg-Dubé syndrome compared with those of Chinese patients with non-BHD diffuse cyst lung diseases.

Authors:  Wenshuai Xu; Zhiyan Xu; Yaping Liu; Yongzhong Zhan; Xin Sui; Ruie Feng; Min Peng; Xue Li; Jun Wang; Shuzhen Meng; Li Wang; Xinlun Tian; Xue Zhang; Kai-Feng Xu
Journal:  Orphanet J Rare Dis       Date:  2020-07-06       Impact factor: 4.123

Review 4.  Childhood pneumothorax in Birt-Hogg-Dubé syndrome: A cohort study and review of the literature.

Authors:  Marianne Geilswijk; Elisabeth Bendstrup; Mia Gebauer Madsen; Mette Sommerlund; Anne-Bine Skytte
Journal:  Mol Genet Genomic Med       Date:  2018-02-13       Impact factor: 2.183

5.  Risk of spontaneous pneumothorax due to air travel and diving in patients with Birt-Hogg-Dubé syndrome.

Authors:  P C Johannesma; I van de Beek; J W T van der Wel; M A Paul; A C Houweling; M A Jonker; J H T M van Waesberghe; R Reinhard; Th M Starink; R J A van Moorselaar; F H Menko; P E Postmus
Journal:  Springerplus       Date:  2016-09-07
  5 in total

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