title: Cystic fibrosis liver disease: to transplant or not to transplant?
abstract: Biliary cirrhosis complicates some adults with cystic fibrosis (CF) and may require transplantation. Cardio-respiratory disease severity varies such that patients may require liver transplantation, heart/lung/liver (triple) grafts or may be too ill for any procedure. A 15-year experience of adults with CF-related liver disease referred for liver transplantation is presented with patient survival as outcome. Twelve patients were listed for triple grafting. Four died of respiratory disease after prolonged waits (4-171 weeks). Eight underwent transplantation (median wait 62 weeks); 5-year actuarial survival was 37.5%. Four died perioperatively; only one is alive at 8-years. Eighteen patients underwent liver transplant alone (median wait 7 weeks); 1- and 5-year actuarial survival rates were 100% and 69%. Three long-term survivors required further organ replacement (two heart/lung and one renal). Two others were turned down for heart/lung transplantation and four have significant renal impairment. Results for triple grafting were poor with unacceptable waiting times. Results for liver transplant alone were satisfactory, with acceptable waiting times and survival. However, further grafts were required and renal impairment was frequent. The policy of early liver transplantation for adults with CF with a view to subsequent heart/lung or renal transplantation needs assessment in the context of long-term outcome.
authors.affiliation: Liver Transplant Unit, Addenbrooke's Hospital, Hills Road, Cambridge, UK. kathryn.nash@addenbrookes.nhs.uk
authors.forename: K L
authors.initials: KL
authors.lastname: Nash
authors.forename: J D
authors.initials: JD
authors.lastname: Collier
authors.forename: J
authors.initials: J
authors.lastname: French
authors.forename: D
authors.initials: D
authors.lastname: McKeon
authors.forename: A E S
authors.initials: AE
authors.lastname: Gimson
authors.forename: N V
authors.initials: NV
authors.lastname: Jamieson
authors.forename: J
authors.initials: J
authors.lastname: Wallwork
authors.forename: D
authors.initials: D
authors.lastname: Bilton
authors.forename: G J M
authors.initials: GJ
authors.lastname: Alexander
mesh_terms: D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications / Q000401:mortality / Q000601:surgery*; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation / Q000401:mortality; D008297:Male; D011183:Postoperative Complications / Q000209:etiology / Q000401:mortality; D012189:Retrospective Studies; D016019:Survival Analysis; D017741:Survivors