title: Lung transplantation for cystic fibrosis: effective and durable therapy in a high-risk group.
abstract: BACKGROUND
  The purpose of this study was to review our experience with lung transplantation in patients with end-stage cystic fibrosis.
  
  
  METHODS
  Eight-two patients with cystic fibrosis have undergone bilateral lung transplantation (n=76) or bilateral lower lobe transplantation (n=6) since October 1990.
  
  
  RESULTS
  Actuarial survival for the entire cohort is 79% at 1 year and 57% at 5 years. The development of bronchiolitis obliterans syndrome is the leading cause of death after the first year. Freedom from bronchiolitis obliterans syndrome is 84% at 1 year and 51% at 3 years. Pulmonary function tests improve dramatically in recipients. There was no association between death within 1 year and recipient age, weight, graft ischemic time, cytomegalovirus seronegativity, or the presence of pan-resistant organisms. Similarly, there was no association between the development of bronchiolitis obliterans syndrome within 2 years and ischemic time, number of rejection episodes, cytomegalovirus seronegativity, or the presence of panresistant organisms.
  
  
  CONCLUSIONS
  Despite their poor nutritional status and the presence of multiply resistant organisms, patients with cystic fibrosis can undergo bilateral lung transplantation with acceptable morbidity and mortality.
authors.affiliation: Division of Cardiothoracic Surgery, University of North Carolina School of Medicine, Chapel Hill 27599-7065, USA.
authors.forename: T M
authors.initials: TM
authors.lastname: Egan
authors.forename: F C
authors.initials: FC
authors.lastname: Detterbeck
authors.forename: M R
authors.initials: MR
authors.lastname: Mill
authors.forename: K K
authors.initials: KK
authors.lastname: Gott
authors.forename: J B
authors.initials: JB
authors.lastname: Rea
authors.forename: J
authors.initials: J
authors.lastname: McSweeney
authors.forename: R M
authors.initials: RM
authors.lastname: Aris
authors.forename: L J
authors.initials: LJ
authors.lastname: Paradowski
mesh_terms: D000293:Adolescent; D000328:Adult; D000758:Anesthesia / Q000379:methods; D001989:Bronchiolitis Obliterans / Q000209:etiology; D002648:Child; D003550:Cystic Fibrosis / Q000401:mortality / Q000601:surgery*; D005260:Female; D006084:Graft Rejection; D006801:Humans; D007165:Immunosuppression Therapy / Q000379:methods; D016040:Lung Transplantation / Q000379:methods; D008297:Male; D008875:Middle Aged; D011182:Postoperative Care / Q000379:methods; D011183:Postoperative Complications; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome