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Pneumothorax in cystic fibrosis

Flume, Patrick A

Current Opinion in Pulmonary Medicine: July 2011 - Volume 17 - Issue 4 - p 220–225
doi: 10.1097/MCP.0b013e328345e1f8
Diseases of the pleura: Edited by Richard W. Light

Purpose of review Cystic fibrosis (CF) is a complex genetic disease affecting many organs, although 85% of the mortality is a result of lung disease. The natural history of the lung disease consists of early and persistent infection, an exaggerated inflammatory response, structural airway changes (i.e. bronchiectasis), and progressive airways obstruction, ultimately resulting in respiratory failure. As airways disease worsens, there is an increased likelihood of respiratory complications, such as pneumothorax, that may be serious. This review describes our current understanding of the pathogenesis of pneumothorax in CF and its treatment.

Recent findings The CF Foundation Pulmonary Therapies Committee recently published their recommendations for the treatment of hemoptysis and pneumothorax. As insufficient data exist from which a systematic review of the literature could be used to develop guidelines, the recommendations were derived from a formalized expert panel consensus process.

Summary We now have recommendations on specific care of the patient with CF who has a pneumothorax.

Departments of Medicine and Pediatrics, Medical University of South Carolina, Charleston, South Carolina, USA

Correspondence to Patrick A. Flume, MD, Medical University of South Carolina, 96 Jonathan Lucas Street, 812-CSB, Charleston, SC 29425, USA Tel: +1 843 792 9219; fax: +1 843 792 0732; e-mail:

© 2011 Lippincott Williams & Wilkins, Inc.