title: Cystic fibrosis chronic rhinosinusitis: a comprehensive review.
abstract: BACKGROUND
  Advances in the care of patients with cystic fibrosis (CF) have improved pulmonary outcomes and survival. In addition, rapid developments regarding the underlying genetic and molecular basis of the disease have led to numerous novel targets for treatment. However, clinical and basic scientific research focusing on therapeutic strategies for CF-associated chronic rhinosinusitis (CRS) lags behind the evidence-based approaches currently used for pulmonary disease.
  
  
  METHODS
  This review evaluates the available literature and provides an update concerning the pathophysiology, current treatment approaches, and future pharmaceutical tactics in the management of CRS in patients with CF.
  
  
  RESULTS
  Optimal medical and surgical strategies for CF CRS are lacking because of a dearth of well-performed clinical trials. Medical and surgical interventions are supported primarily by level 2 or 3 evidence and are aimed at improving clearance of mucus, infection, and inflammation. A number of novel therapeutics that target the basic defect in the cystic fibrosis transmembrane conductance regulator channel are currently under investigation. Ivacaftor, a corrector of the G551D mutation, was recently approved by the Food and Drug Administration. However, sinonasal outcomes using this and other novel drugs are pending.
  
  
  CONCLUSIONS
  CRS is a lifelong disease in CF patients that can lead to substantial morbidity and decreased quality of life. A multidisciplinary approach will be necessary to develop consistent and evidence-based treatment paradigms.
authors.affiliation: Department of Surgery/Division of Otolaryngology, University of Alabama at Birmingham, Birmingham, Alabama, USA.
authors.forename: Mohamad R
authors.initials: MR
authors.lastname: Chaaban
authors.forename: Alexandra
authors.initials: A
authors.lastname: Kejner
authors.forename: Steven M
authors.initials: SM
authors.lastname: Rowe
authors.forename: Bradford A
authors.initials: BA
authors.lastname: Woodworth
mesh_terms: D000627:Aminophenols / Q000627:therapeutic use; D000818:Animals; D002908:Chronic Disease; D002986:Clinical Trials as Topic; D003550:Cystic Fibrosis / Q000150:complications / Q000276:immunology* / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism; D019317:Evidence-Based Medicine; D006801:Humans; D015363:Quinolones / Q000627:therapeutic use; D012220:Rhinitis / Q000150:complications / Q000276:immunology* / Q000628:therapy; D012852:Sinusitis / Q000150:complications / Q000276:immunology* / Q000628:therapy; D014481:United States; D014486:United States Food and Drug Administration
chemical_list: D000627:Aminophenols; D015363:Quinolones; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; C545203:ivacaftor