A Young Man with Bilateral Spontaneous Pneumothorax

Case. A 33-year-old male nonsmoker presented with sudden onset of dyspnoea and thoracic pain. Chest radiograph showed a left-sided pneumothorax. Few days later he developed a right-sided pneumothorax. He had a positive family history of pneumothorax. High-resolution computed tomography of the chest...

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Main Author: Liese Lieve Willemien Verhaert
Format: Article
Language:English
Published: Wiley 2011-01-01
Series:Case Reports in Pulmonology
Online Access:http://dx.doi.org/10.1155/2011/414165
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author Liese Lieve Willemien Verhaert
author_facet Liese Lieve Willemien Verhaert
author_sort Liese Lieve Willemien Verhaert
collection DOAJ
description Case. A 33-year-old male nonsmoker presented with sudden onset of dyspnoea and thoracic pain. Chest radiograph showed a left-sided pneumothorax. Few days later he developed a right-sided pneumothorax. He had a positive family history of pneumothorax. High-resolution computed tomography of the chest showed multiple pulmonary cysts predominantly located in the lower lung regions. We suspected Birt-Hogg-Dubé syndrome (BHD). Conclusion. Birt-Hogg-Dubé syndrome is a rare autosomal dominant inherited genodermatosis and characterised by clinical manifestations including hamartomas of the skin, renal tumors, and pulmonary cysts with spontaneous pneumothorax. BHD is probably underdiagnosed because of the wide variability in its clinical expression. It is important to recognize these patients because of the possibility of developing renal cancer.
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spelling doaj-art-cabb70b6344441e0a8ae4608e0bf47c82025-08-20T03:24:04ZengWileyCase Reports in Pulmonology2090-68462090-68542011-01-01201110.1155/2011/414165414165A Young Man with Bilateral Spontaneous PneumothoraxLiese Lieve Willemien Verhaert0Catharina Hospital, Eindhoven, The NetherlandsCase. A 33-year-old male nonsmoker presented with sudden onset of dyspnoea and thoracic pain. Chest radiograph showed a left-sided pneumothorax. Few days later he developed a right-sided pneumothorax. He had a positive family history of pneumothorax. High-resolution computed tomography of the chest showed multiple pulmonary cysts predominantly located in the lower lung regions. We suspected Birt-Hogg-Dubé syndrome (BHD). Conclusion. Birt-Hogg-Dubé syndrome is a rare autosomal dominant inherited genodermatosis and characterised by clinical manifestations including hamartomas of the skin, renal tumors, and pulmonary cysts with spontaneous pneumothorax. BHD is probably underdiagnosed because of the wide variability in its clinical expression. It is important to recognize these patients because of the possibility of developing renal cancer.http://dx.doi.org/10.1155/2011/414165
spellingShingle Liese Lieve Willemien Verhaert
A Young Man with Bilateral Spontaneous Pneumothorax
Case Reports in Pulmonology
title A Young Man with Bilateral Spontaneous Pneumothorax
title_full A Young Man with Bilateral Spontaneous Pneumothorax
title_fullStr A Young Man with Bilateral Spontaneous Pneumothorax
title_full_unstemmed A Young Man with Bilateral Spontaneous Pneumothorax
title_short A Young Man with Bilateral Spontaneous Pneumothorax
title_sort young man with bilateral spontaneous pneumothorax
url http://dx.doi.org/10.1155/2011/414165
work_keys_str_mv AT lieselievewillemienverhaert ayoungmanwithbilateralspontaneouspneumothorax
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