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BMC Med. 2012; 10: 88.
Published online Aug 10, 2012. doi:  10.1186/1741-7015-10-88
PMCID: PMC3425089
State of progress in treating cystic fibrosis respiratory disease
Patrick A Flumecorresponding author1 and Donald R Van Devanter2
1Medical University of South Carolina, Charleston, SC, USA
2Case Western Reserve University School of Medicine, Cleveland, OH, USA
corresponding authorCorresponding author.
Patrick A Flume: flumepa/at/musc.edu; Donald R Van Devanter: enigmaster/at/comcast.net
Received June 2, 2012; Accepted August 10, 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
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