title: Outcomes of lung transplantation for cystic fibrosis in a large UK cohort.
abstract: BACKGROUND
  Lung transplantation is an important option to treat patients with advanced cystic fibrosis (CF) lung disease. The outcomes of a large UK cohort of CF lung transplantation recipients is reported.
  
  
  METHODS
  Retrospective review of case notes and transplantation databases.
  
  
  RESULTS
  176 patients with CF underwent lung transplantation at our centre. The majority (168) had bilateral sequential lung transplantation. Median age at transplantation was 26 years. Diabetes was common pretransplantation (40%). Polymicrobial infection was common in individual recipients. A diverse range of pathogens were encountered, including the Burkholderia cepacia complex (BCC). The bronchial anastomotic complication rate was 2%. Pulmonary function (forced expiratory volume in 1 s % predicted) improved from a pretransplantation median of 0.8 l (21% predicted) to 2.95 l (78% predicted) at 1 year following transplantation. We noted an acute rejection rate of 41% within the first month. Our survival values were 82% survival at 1 year, 70% at 3 years, 62% at 5 years and 51% at 10 years. Patients with BCC infection had poorer outcomes and represented the majority of those who had a septic death. Data are presented on those free from these infections. Bronchiolitis obliterans syndrome (BOS) and sepsis were common causes of death. Freedom from BOS was 74% at 5 years and 38% at 10 years. Biochemical evidence of renal dysfunction was common although renal replacement was infrequently required (<5%).
  
  
  CONCLUSIONS
  Lung transplantation is an important therapeutic option in patients with CF even in those with more complex microbiology. Good functional outcomes are noted although transplantation associated morbidities accrue with time.
authors.affiliation: Department of Cardiopulmonary Transplantation, The Freeman Hospital, High Heaton, Newcastle-upon-Tyne, UK.
authors.forename: G
authors.initials: G
authors.lastname: Meachery
authors.forename: A
authors.initials: A
authors.lastname: De Soyza
authors.forename: A
authors.initials: A
authors.lastname: Nicholson
authors.forename: G
authors.initials: G
authors.lastname: Parry
authors.forename: A
authors.initials: A
authors.lastname: Hasan
authors.forename: K
authors.initials: K
authors.lastname: Tocewicz
authors.forename: T
authors.initials: T
authors.lastname: Pillay
authors.forename: S
authors.initials: S
authors.lastname: Clark
authors.forename: J L
authors.initials: JL
authors.lastname: Lordan
authors.forename: S
authors.initials: S
authors.lastname: Schueler
authors.forename: A J
authors.initials: AJ
authors.lastname: Fisher
authors.forename: J H
authors.initials: JH
authors.lastname: Dark
authors.forename: F K
authors.initials: FK
authors.lastname: Gould
authors.forename: P A
authors.initials: PA
authors.lastname: Corris
mesh_terms: D000293:Adolescent; D000328:Adult; D000402:Airway Obstruction / Q000401:mortality; D001989:Bronchiolitis Obliterans / Q000401:mortality; D001992:Bronchoalveolar Lavage Fluid / Q000382:microbiology; D002648:Child; D003550:Cystic Fibrosis / Q000382:microbiology / Q000401:mortality / Q000601:surgery*; D048909:Diabetes Complications / Q000401:mortality; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology / Q000401:mortality; D016040:Lung Transplantation / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000401:mortality; D011183:Postoperative Complications / Q000209:etiology* / Q000401:mortality; D011300:Preoperative Care; D006435:Renal Dialysis / Q000706:statistics & numerical data; D012086:Reoperation; D013183:Sputum / Q000382:microbiology; D006113:United Kingdom / Q000453:epidemiology