Document Detail


Recurrent spontaneous pneumothorax in a patient with Birt-Hogg-Dubé syndrome.
MedLine Citation:
PMID:  20692178     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Recurrent spontaneous pneumothorax is a disorder often managed by thoracic surgeons. Most etiologies are benign in nature; however, there are several syndromes that are associated with potentially fatal pulmonary or systemic manifestations. One such example is Birt-Hogg-Dubé syndrome, a rare inheritable syndrome characterized by papular skin lesions involving the scalp, head, face and neck; pulmonary cysts; and a propensity to develop renal carcinoma. In our report, we describe a patient, who was diagnosed with Birt-Hogg-Dubé syndrome after presenting with a history of recurrent spontaneous pneumothorax, and a family history of spontaneous pneumothorax and renal cell carcinoma. This case is of particular interest to the cardiothoracic surgery community as the patient described as well as several of his family members were managed by multiple thoracic surgeons, who did not appreciate the diagnosis.
Authors:
Jarrod D Predina; Robert M Kotloff; Wallace T Miller; Sunil Singhal
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Publication Detail:
Type:  Journal Article     Date:  2010-08-06
Journal Detail:
Title:  European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery     Volume:  39     ISSN:  1873-734X     ISO Abbreviation:  Eur J Cardiothorac Surg     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-02-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8804069     Medline TA:  Eur J Cardiothorac Surg     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  404-6     Citation Subset:  IM    
Copyright Information:
Crown Copyright © 2010. Published by Elsevier B.V. All rights reserved.
Affiliation:
Division of Thoracic Surgery, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
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