Birt-Hogg-Dubé syndrome in apparent primary spontaneous pneumothorax patients; results and recommendations for clinical practice

Birt-Hogg-Dubé syndrome in apparent primary spontaneous pneumothorax patients; results and recommendations for clinical practice

Abstract Background Birt-Hogg-Dubé syndrome (BHD) is an inherited disease caused by pathogenic variants in the FLCN gene. One of the characteristics is the increased risk for spontaneous pneumothorax, likely due to the presence of pulmonary cysts mainly distributed under the carina. Due to variable...

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Bibliographic Details
Main Authors: Jincey D. Sriram, Irma van de Beek, Paul C. Johannesma, Michiel H. van Werkum, Tijmen J. W. T. van der Wel, Elise M. Wessels, Hans J. J. P. Gille, Arjan C. Houweling, Pieter E. Postmus, Hans J. M. Smit
Format: Article
Language:English
Published: BMC 2022-08-01
Series:BMC Pulmonary Medicine
Subjects:
BHD
Birt-Hogg-Dubé syndrome
Pneumothorax
Primary spontaneous pneumothorax
PSP
Online Access:https://doi.org/10.1186/s12890-022-02107-7

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