title: Transplantation for cystic fibrosis: outcome following early liver transplantation.
abstract: OBJECTIVE
  Life expectancy in patients with cystic fibrosis (CF) has recently improved due to numerous factors, including a multidisciplinary approach to their management. Prolonged survival may have led to an increasing impact of liver disease on the prognosis of CF patients. The aim of this study was to assess the role of liver transplantation in patients with CF.
  
  
  METHODS
  The factors influencing outcome in 24 patients (15 adults and nine children) with CF who have received single liver transplantation, triple heart-lung-liver transplantation (tx) or died while being assessed for triple grafting, were analyzed.
  
  
  RESULTS
  Median age at tx in single liver recipients (13 years) was lower than in triple graft recipients (21 years) and those who died (23 years). All patients who received single liver tx made an excellent recovery, including significant improvement of their respiratory function (mean forced vital capacity (FVC) increased from 61% before transplantation to 82% of expected, 6-9 months after tx). Four out of five patients who received triple tx died (0-2 months) after operation. On the basis of our retrospective review, we propose modifications to an existing scoring system for liver tx assessment in CF by scoring additional points for elevated white blood count, bilirubin, and impaired pulmonary function. These changes will need to be evaluated prospectively to confirm their predictive value.
  
  
  CONCLUSIONS
  Liver transplantation is effective therapy in young patients with cystic fibrosis, portal hypertension and hepatic dysfunction, and is indicated before a critical stage of deteriorating lung function is reached. In patients with both end-stage liver and lung disease, triple tx has a poor prognosis. Pre-emptive liver tx in younger patients with CF not only has a better outcome but improves lung function.
authors.affiliation: Liver and Hepatobiliary Unit, Queen Elizabeth Hospital, Edgbaston, Birmiongham, UK.
authors.forename: Piotr
authors.initials: P
authors.lastname: Milkiewicz
authors.forename: Grzegorz
authors.initials: G
authors.lastname: Skiba
authors.forename: Deidre
authors.initials: D
authors.lastname: Kelly
authors.forename: Peter
authors.initials: P
authors.lastname: Weller
authors.forename: Robert
authors.initials: R
authors.lastname: Bonser
authors.forename: Ufuk
authors.initials: U
authors.lastname: Gur
authors.forename: Darius
authors.initials: D
authors.lastname: Mirza
authors.forename: John
authors.initials: J
authors.lastname: Buckels
authors.forename: David
authors.initials: D
authors.lastname: Stableforth
authors.forename: Elwyn
authors.initials: E
authors.lastname: Elias
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D004932:Esophageal and Gastric Varices / Q000150:complications; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications; D008107:Liver Diseases / Q000150:complications / Q000601:surgery*; D016031:Liver Transplantation; D016040:Lung Transplantation; D008297:Male; D011379:Prognosis; D015656:Respiratory Mechanics; D012189:Retrospective Studies; D013163:Splenomegaly / Q000150:complications