title: Cystic fibrosis in adults: current and future management strategies.
abstract: Over 30,000 individuals in the United States of America are living with cystic fibrosis (CF). Despite incremental advances in care and understanding of its pathophysiology, CF remains a significantly life-limiting disease. Readily accessible newborn screening, genetic testing, and an improved awareness have increased the early recognition of CF, atypical presentations of CF, and the CF-related diseases. Improvements in medical management have led to continually improving life expectancy for patients with CF. Despite improved management strategies, severe lung disease remains the commonly life-limiting pathology. We review the pathophysiology, diagnosis, and management of the respiratory-tract manifestations of CF that represent the life-limiting aspects of the condition and summarize upcoming and possible future therapies for patients with CF.
authors.affiliation: Adult Cystic Fibrosis Clinic and University of California Davis School of Medicine, Sacramento, CA, USA. bmmorrissey@ucdavis.edu
authors.forename: Brian M
authors.initials: BM
authors.lastname: Morrissey
authors.forename: Bettina C
authors.initials: BC
authors.lastname: Schock
authors.forename: Gregory P
authors.initials: GP
authors.lastname: Marelich
authors.forename: Carroll E
authors.initials: CE
authors.lastname: Cross
mesh_terms: D000328:Adult; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy* / Q000821:virology; D004783:Environmental Microbiology; D015316:Genetic Therapy; D006801:Humans; D016040:Lung Transplantation; D016106:Methicillin Resistance