title: Liver disease in adult patients with cystic fibrosis: a frequent and independent prognostic factor associated with death or lung transplantation.
abstract: OBJECTIVE
  Increased life expectancy in patients with cystic fibrosis (CF) allows better knowledge of non-pulmonary complications like liver disease (CFLD). However, few data have been published in large adult cohorts. The aim of this study was to estimate the prevalence and the prognosis of CFLD in adult CF patients.
  
  
  METHODS
  A retrospective analysis of a monocentric cohort of adult CF patients prospectively followed, at least every year, was performed. CFLD was diagnosed using published composite criteria. If cirrhosis was suspected, upper digestive endoscopy was realized to assess the presence of portal hypertension.
  
  
  RESULTS
  A cohort of 285 adult CF patients was followed during 4.8 ± 3.6 years. Among them, 90 had CFLD at the beginning of follow-up and 23 a suspicion of cirrhosis. Factors independently associated with liver disease at baseline were history of meconium ileus, pancreatic insufficiency, chronic colonization with Burkholderia cepacia and the number of IV antibiotic courses per year. Nine patients developed liver decompensation during follow-up, all with a suspicion of cirrhosis at baseline. Six patients underwent liver transplantation alone and three patients combined liver and lung transplantation. Factors independently associated with death or lung transplantation at baseline were liver disease, BMI, forced expiratory volume in 1 second and number of IV antibiotic courses per year.
  
  
  CONCLUSIONS
  CFLD was present at baseline in one third of adult patients with CF with a marked risk of liver decompensation during follow-up. Moreover, CFLD at baseline appears as an independent factor associated with death or lung transplantation.
authors.affiliation: Université Paris-Descartes, Paris, France.
authors.forename: Ariane
authors.initials: A
authors.lastname: Chryssostalis
authors.forename: Dominique
authors.initials: D
authors.lastname: Hubert
authors.forename: Joël
authors.initials: J
authors.lastname: Coste
authors.forename: Reem
authors.initials: R
authors.lastname: Kanaan
authors.forename: Pierre-Régis
authors.initials: PR
authors.lastname: Burgel
authors.forename: Nadine
authors.initials: N
authors.lastname: Desmazes-Dufeu
authors.forename: Olivier
authors.initials: O
authors.lastname: Soubrane
authors.forename: Daniel
authors.initials: D
authors.lastname: Dusser
authors.forename: Philippe
authors.initials: P
authors.lastname: Sogni
mesh_terms: D000328:Adult; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000150:complications* / Q000401:mortality / Q000503:physiopathology / Q000601:surgery; D018572:Disease-Free Survival; D005260:Female; D005541:Forced Expiratory Volume; D005602:France / Q000453:epidemiology; D006801:Humans; D053208:Kaplan-Meier Estimate; D008107:Liver Diseases / Q000209:etiology* / Q000401:mortality / Q000601:surgery; D016031:Liver Transplantation; D016040:Lung Transplantation; D008297:Male; D015999:Multivariate Analysis; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors