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Open Access Review

State of progress in treating cystic fibrosis respiratory disease

Patrick A Flume1* and Donald R Van Devanter2

Author Affiliations

1 Medical University of South Carolina, Charleston, SC, USA

2 Case Western Reserve University School of Medicine, Cleveland, OH, USA

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BMC Medicine 2012, 10:88  doi:10.1186/1741-7015-10-88

Published: 10 August 2012

Abstract

Since the discovery of the gene associated with cystic fibrosis (CF), there has been tremendous progress in the care of patients with this disease. New therapies have entered the market and are part of the standard treatment of patients with CF, and have been associated with marked improvement in survival. Now there are even more promising therapies directed at different components of the pathophysiology of this disease. In this review, our current knowledge of the pathophysiology of lung disease in patients with CF is described, along with the current treatment of CF lung disease, and the therapies in development that offer great promise to our patients.

Keywords:
cystic fibrosis; pathophysiology; treatment