title: Cystic Fibrosis Associated with Worse Survival After Liver Transplantation.
abstract: BACKGROUND
  Survival in cystic fibrosis patients after liver transplantation and liver-lung transplantation is not well studied.
  
  
  OBJECTIVE
  To discern survival rates after liver transplantation and liver-lung transplantation in patients with and without cystic fibrosis.
  
  
  METHODS
  The United Network for Organ Sharing database was queried from 1987 to 2013. Univariate Cox proportional hazards, multivariate Cox models, and propensity score matching were performed.
  
  
  RESULTS
  Liver transplant and liver-lung transplant were performed in 212 and 53 patients with cystic fibrosis, respectively. Univariate Cox proportional hazards regression identified lower survival in cystic fibrosis after liver transplant compared to a reference non-cystic fibrosis liver transplant cohort (HR 1.248; 95 % CI 1.012, 1.541; p = 0.039). Supplementary analysis found graft survival was similar across the 3 recipient categories (log-rank test: χ(2) 2.68; p = 0.262). Multivariate Cox models identified increased mortality hazard among cystic fibrosis patients undergoing liver transplantation (HR 2.439; 95 % CI 1.709, 3.482; p < 0.001) and liver-lung transplantation (HR 2.753; 95 % CI 1.560, 4.861; p < 0.001). Propensity score matching of cystic fibrosis patients undergoing liver transplantation to non-cystic fibrosis controls identified a greater mortality hazard in the cystic fibrosis cohort using a Cox proportional hazards model stratified on matched pairs (HR 3.167; 95 % CI 1.265, 7.929, p = 0.014).
  
  
  CONCLUSIONS
  Liver transplantation in cystic fibrosis is associated with poorer long-term patient survival compared to non-cystic fibrosis patients, although the difference is not due to graft survival.
authors.affiliation: Department of Surgery, The Ohio State University College of Medicine, Columbus, OH, USA.|Center for Epidemiology of Organ Failure and Transplantation, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, Columbus, OH, 43205, USA.
authors.forename: Sylvester M
authors.initials: SM
authors.lastname: Black
authors.affiliation: Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, USA.|Center for Epidemiology of Organ Failure and Transplantation, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, Columbus, OH, 43205, USA.|Section of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, 43205, USA.
authors.forename: Frederick W
authors.initials: FW
authors.lastname: Woodley
authors.affiliation: Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, USA.|Center for Epidemiology of Organ Failure and Transplantation, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, Columbus, OH, 43205, USA.|Department of Anesthesiology and Pain Medicine, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, Columbus, OH, 43205, USA.
authors.forename: Dmitry
authors.initials: D
authors.lastname: Tumin
authors.affiliation: Department of Internal Medicine, The Ohio State University College of Medicine, Columbus, OH, USA.|Center for Epidemiology of Organ Failure and Transplantation, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, Columbus, OH, 43205, USA.
authors.forename: Khalid
authors.initials: K
authors.lastname: Mumtaz
authors.affiliation: Department of Surgery, The Ohio State University College of Medicine, Columbus, OH, USA.|Center for Epidemiology of Organ Failure and Transplantation, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, Columbus, OH, 43205, USA.
authors.forename: Bryan A
authors.initials: BA
authors.lastname: Whitson
authors.affiliation: Department of Anesthesiology, The Ohio State University College of Medicine, Columbus, OH, USA.|Center for Epidemiology of Organ Failure and Transplantation, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, Columbus, OH, 43205, USA.|Department of Anesthesiology and Pain Medicine, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, Columbus, OH, 43205, USA.
authors.forename: Joseph D
authors.initials: JD
authors.lastname: Tobias
authors.affiliation: Department of Surgery, The Ohio State University College of Medicine, Columbus, OH, USA. hayes.705@osu.edu.|Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, USA. hayes.705@osu.edu.|Department of Internal Medicine, The Ohio State University College of Medicine, Columbus, OH, USA. hayes.705@osu.edu.|Center for Epidemiology of Organ Failure and Transplantation, Nationwide Children's Hospital, The Ohio State University, 700 Children's Drive, Columbus, OH, 43205, USA. hayes.705@osu.edu.|Section of Pulmonary Medicine, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, 43205, USA. hayes.705@osu.edu.
authors.forename: Don
authors.initials: D
authors.lastname: Hayes
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000601:surgery; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D017093:Liver Failure / Q000150:complications* / Q000601:surgery; D016031:Liver Transplantation / Q000401:mortality*; D016040:Lung Transplantation / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D057216:Propensity Score; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D014481:United States / Q000453:epidemiology; D055815:Young Adult
keywords: Cystic fibrosis; Liver transplantation; Lung transplantation; Survival