title: Lung transplantation for cystic fibrosis: differential characteristics and outcomes between children and adults.
abstract: OBJECTIVE
  The survival benefit of lung transplantation (LTx) for cystic fibrosis (CF) patients is well demonstrated. We aim to compare children and adult CF recipients to assess whether there are differences in survival and clinical outcomes, and to identify risk factors for mortality.
  
  
  METHODS
  A retrospective analysis of 442 consecutive LTx performed at our institution in a 20-year period was conducted. CF patients were distributed into two groups: children (age <18 years) and adults (age ≥18 years). Donor and recipient general demographic data, perioperative and postoperative factors including 30-day mortality, survival, primary graft dysfunction (PGD), complications, acute rejection (AR) and chronic lung allograft dysfunction (CLAD) were analysed and compared between groups. Univariable, Kaplan-Meier and Cox regression analyses were performed.
  
  
  RESULTS
  The study group included 120 consecutive CF patients: 50 children (13 ± 3 years) and 70 adults (25 ± 6 years) undergoing 111 bilateral, 4 lobar, 4 combined and 1 unilateral LTx. Comparative analysis (children versus adults): survival (overall; 5, 10 and 15 years) 57, 45, 35% vs 67, 55, 43% (P = 0.32); survival (1-year survivors; 5, 10 and 15 years): 75, 64, 46% vs 90, 75, 59% (P = 0.09); 30-day mortality: 14 vs 16% (P = 0.27); urgent LTx: 32 vs 17% (P = 0.04); use of cardiopulmonary bypass (CPB): 56 vs 28% (P = 0.002); intensive care unit stay: 20 ± 19 vs 10 ± 9 days (P = 0.006); AR episodes (n): 1.4 ± 0.7 vs 1.2 ± 0.8 (P = 0.004). Incidence of PGD and freedom from CLAD did not differ between groups. Predictors of mortality were: use of CPB (HR 3.12; 95% CI 1.33-7.35; P < 0.01), post-transplant diabetes mellitus (HR 2.49; 95% CI 1.13-5.43; P = 0.02) and pneumonia episodes within the first month post-transplant (HR 2.82; 95% CI 1.27-6.29; P = 0.01).
  
  
  CONCLUSIONS
  Paediatric CF patients usually present with poorer pre-transplant status, require CPB more frequently and have a higher incidence of post-LTx diabetes and infections. This might explain the trend towards a better long-term survival observed in adult CF patients.
authors.affiliation: Thoracic Surgery and Lung Transplantation Unit, University Hospital Reina Sofia, Córdoba, Spain Group for the Study of Thoracic Neoplasms and Lung Transplantation, IMIBIC (Instituto Maimónides de Investigación Biomédica de Córdoba), University of Córdoba, Córdoba, Spain pmoreno39@gmail.com.
authors.forename: Paula
authors.initials: P
authors.lastname: Moreno
authors.affiliation: Thoracic Surgery and Lung Transplantation Unit, University Hospital Reina Sofia, Córdoba, Spain Group for the Study of Thoracic Neoplasms and Lung Transplantation, IMIBIC (Instituto Maimónides de Investigación Biomédica de Córdoba), University of Córdoba, Córdoba, Spain.
authors.forename: Antonio
authors.initials: A
authors.lastname: Alvarez
authors.affiliation: Thoracic Surgery and Lung Transplantation Unit, University Hospital Reina Sofia, Córdoba, Spain Group for the Study of Thoracic Neoplasms and Lung Transplantation, IMIBIC (Instituto Maimónides de Investigación Biomédica de Córdoba), University of Córdoba, Córdoba, Spain.
authors.forename: Guadalupe
authors.initials: G
authors.lastname: Carrasco
authors.affiliation: Thoracic Surgery and Lung Transplantation Unit, University Hospital Reina Sofia, Córdoba, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Redel
authors.affiliation: Thoracic Surgery and Lung Transplantation Unit, University Hospital Reina Sofia, Córdoba, Spain Group for the Study of Thoracic Neoplasms and Lung Transplantation, IMIBIC (Instituto Maimónides de Investigación Biomédica de Córdoba), University of Córdoba, Córdoba, Spain.
authors.forename: Hugo Dario
authors.initials: HD
authors.lastname: Guaman
authors.affiliation: Thoracic Surgery and Lung Transplantation Unit, University Hospital Reina Sofia, Córdoba, Spain Group for the Study of Thoracic Neoplasms and Lung Transplantation, IMIBIC (Instituto Maimónides de Investigación Biomédica de Córdoba), University of Córdoba, Córdoba, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Baamonde
authors.affiliation: Thoracic Surgery and Lung Transplantation Unit, University Hospital Reina Sofia, Córdoba, Spain Group for the Study of Thoracic Neoplasms and Lung Transplantation, IMIBIC (Instituto Maimónides de Investigación Biomédica de Córdoba), University of Córdoba, Córdoba, Spain.
authors.forename: Francisco Javier
authors.initials: FJ
authors.lastname: Algar
authors.affiliation: Thoracic Surgery and Lung Transplantation Unit, University Hospital Reina Sofia, Córdoba, Spain Group for the Study of Thoracic Neoplasms and Lung Transplantation, IMIBIC (Instituto Maimónides de Investigación Biomédica de Córdoba), University of Córdoba, Córdoba, Spain.
authors.forename: Francisco
authors.initials: F
authors.lastname: Cerezo
authors.affiliation: Thoracic Surgery and Lung Transplantation Unit, University Hospital Reina Sofia, Córdoba, Spain Group for the Study of Thoracic Neoplasms and Lung Transplantation, IMIBIC (Instituto Maimónides de Investigación Biomédica de Córdoba), University of Córdoba, Córdoba, Spain.
authors.forename: Angel
authors.initials: A
authors.lastname: Salvatierra
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003550:Cystic Fibrosis / Q000453:epidemiology / Q000401:mortality / Q000601:surgery*; D005260:Female; D006801:Humans; D016040:Lung Transplantation / Q000009:adverse effects / Q000401:mortality / Q000706:statistics & numerical data; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
keywords: Adults; Cystic fibrosis; Lung transplantation; Outcomes; Paediatrics