The Pathogenesis of Pneumothorax in Birt-Hogg-Dubé Syndrome: a Hypothesis

Overview

Journal Respirology

Specialty Pulmonary Medicine

Date 2014 Oct 11

PMID 25302759

Citations 11

Authors

Paul Christiaan Johannesma

Arjan C Houweling

Jan-Hein T M van Waesberghe

R J Jeroen A van Moorselaar

Theo M Starink

Fred H Menko

Pieter E Postmus

Affiliations

Soon will be listed here.

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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