title: State of progress in treating cystic fibrosis respiratory disease.
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.
authors.affiliation: Medical University of South Carolina, Charleston, SC, USA. flumepa@musc.edu
authors.forename: Patrick A
authors.initials: PA
authors.lastname: Flume
authors.forename: Donald R
authors.initials: DR
authors.lastname: Van Devanter
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003550:Cystic Fibrosis / Q000188:drug therapy / Q000503:physiopathology* / Q000601:surgery / Q000628:therapy*; D015316:Genetic Therapy; D006801:Humans
chemical_list: D000900:Anti-Bacterial Agents; D000893:Anti-Inflammatory Agents