title: Survival in Patients with Advanced Non-cystic Fibrosis Bronchiectasis Versus Cystic Fibrosis on the Waitlist for Lung Transplantation.
abstract: BACKGROUND
  Survival in non-cystic fibrosis (CF) bronchiectasis is not well studied.
  
  
  METHODS
  The United Network for Organ Sharing database was queried from 1987 to 2013 to compare survival in adult patients with non-CF bronchiectasis to patients with CF listed for lung transplantation (LTx). Each subject was tracked from waitlist entry date until death or censoring to determine survival differences between the two groups.
  
  
  RESULTS
  Of 2112 listed lung transplant candidates with bronchiectasis (180 non-CF, 1932 CF), 1617 were used for univariate Cox and Kaplan-Meier survival function analysis, 1173 for multivariate Cox models, and 182 for matched-pairs analysis based on propensity scores. Compared to CF, patients with non-CF bronchiectasis had a significantly lower mortality by univariate Cox analysis (HR 0.565; 95 % CI 0.424, 0.754; p < 0.001). Adjusting for potential confounders, multivariate Cox models identified a significant reduction in risk for death associated with non-CF bronchiectasis who were lung transplant candidates (HR 0.684; 95 % CI 0.475, 0.985; p = 0.041). Results were consistent in multivariate models adjusting for pulmonary hypertension and forced expiratory volume in one second.
  
  
  CONCLUSIONS
  Non-CF bronchiectasis with advanced lung disease was associated with significantly lower mortality hazard compared to CF bronchiectasis on the waitlist for LTx. Separate referral and listing criteria for LTx in non-CF and CF populations should be considered.
authors.affiliation: Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, 43205, Columbus, OH, USA. hayes.705@osu.edu.|Department of Internal Medicine, The Ohio State University, Columbus, OH, USA. hayes.705@osu.edu.|Department of Surgery, The Ohio State University, Columbus, OH, USA. hayes.705@osu.edu.|Section of Pulmonary Medicine, Nationwide Children's Hospital, Columbus, OH, USA. hayes.705@osu.edu.
authors.forename: Don
authors.initials: D
authors.lastname: Hayes
authors.affiliation: Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, 43205, Columbus, OH, USA.|Section of Pulmonary Medicine, Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Benjamin T
authors.initials: BT
authors.lastname: Kopp
authors.affiliation: Department of Anesthesiology, The Ohio State University, Columbus, OH, USA.|Department of Anesthesiology and Pain Medicine, Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Joseph D
authors.initials: JD
authors.lastname: Tobias
authors.affiliation: Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, 43205, Columbus, OH, USA.|Section of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Frederick W
authors.initials: FW
authors.lastname: Woodley
authors.affiliation: Skaggs Center of Pharmaceutical Sciences, The University of Arizona, Tucson, AZ, USA.
authors.forename: Heidi M
authors.initials: HM
authors.lastname: Mansour
authors.affiliation: Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, 43205, Columbus, OH, USA.|Department of Anesthesiology and Pain Medicine, Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Dmitry
authors.initials: D
authors.lastname: Tumin
authors.affiliation: Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, 43205, Columbus, OH, USA.|Department of Internal Medicine, The Ohio State University, Columbus, OH, USA.|Section of Pulmonary Medicine, Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Stephen E
authors.initials: SE
authors.lastname: Kirkby
mesh_terms: D000328:Adult; D001987:Bronchiectasis / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery; D003550:Cystic Fibrosis / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D015996:Survival Rate; D014481:United States / Q000453:epidemiology; D014850:Waiting Lists / Q000401:mortality*; D055815:Young Adult
keywords: Bronchiectasis; Cystic fibrosis; Non-cystic fibrosis; Survival