title: Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health.
abstract: Cystic fibrosis (CF) is an autosomal recessive disease characterized by abnormal airways secretions, chronic endobronchial infection, and progressive airway obstruction. The use of medications to slow the progression of lung disease has led to significant improvement in survival. An evidence review of chronic medications for CF lung disease was performed in 2007 to provide guidance to clinicians in evaluating and selecting appropriate treatment for individuals with this disease. We have undertaken a new review of the literature to update the recommendations, including consideration of new medications and additional evidence on previously reviewed therapies. A multidisciplinary committee of experts in CF pulmonary care was established to review the evidence for use of chronic medications for CF lung disease and make treatment recommendations. Published evidence for chronic lung therapies was systematically reviewed and resulting treatment recommendations were graded based on the United States Preventive Services Task Force scheme. These guidelines provide up-to-date evidence of safety and efficacy of chronic treatments of CF lung disease, including the use of novel therapies that have not previously been included in CF pulmonary guidelines.
authors.affiliation: Department of Pediatrics, The Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA. pmogayze@jhmi.edu
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Mogayzel
authors.forename: Edward T
authors.initials: ET
authors.lastname: Naureckas
authors.forename: Karen A
authors.initials: KA
authors.lastname: Robinson
authors.forename: Gary
authors.initials: G
authors.lastname: Mueller
authors.forename: Denis
authors.initials: D
authors.lastname: Hadjiliadis
authors.forename: Jeffrey B
authors.initials: JB
authors.lastname: Hoag
authors.forename: Lisa
authors.initials: L
authors.lastname: Lubsch
authors.forename: Leslie
authors.initials: L
authors.lastname: Hazle
authors.forename: Kathy
authors.initials: K
authors.lastname: Sabadosa
authors.forename: Bruce
authors.initials: B
authors.lastname: Marshall
mesh_terms: D000318:Adrenergic beta-Agonists / Q000627:therapeutic use*; D000890:Anti-Infective Agents / Q000627:therapeutic use*; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D001993:Bronchodilator Agents / Q000627:therapeutic use*; D003550:Cystic Fibrosis / Q000188:drug therapy*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000819:agonists; D019317:Evidence-Based Medicine; D006801:Humans; D017410:Practice Guidelines as Topic
chemical_list: D000318:Adrenergic beta-Agonists; D000890:Anti-Infective Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D001993:Bronchodilator Agents; D019005:Cystic Fibrosis Transmembrane Conductance Regulator