title: Outcome in patients with cystic fibrosis liver disease.
abstract: BACKGROUND
  Liver disease is an important complication in CF.
  
  
  OBJECTIVE
  To determine if CFLD is a risk factor for mortality in CF, and which baseline characteristics predict all-cause mortality.
  
  
  METHODS
  Irish children with CFLD, and their age and gender matched controls were enrolled at baseline and reviewed after 10years to determine which characteristics predict mortality.
  
  
  RESULTS
  72/84 (85.71%) participants were followed, (mean age Cases 21.71yrs SD 6.5, CF controls 23.62 SD 5.6, 22 (61%) males), with no difference in duration of follow-up. Nineteen participants (26.4%) died, 38.9% (14/36) with CFLD and 13.89% (5/36) CF controls (Odds Ratio (OR) 3.94 95% CI:1.23-12.56 p=0.005). In logistic regression, liver disease (OR 4.28 95% CI 1.07-17.16) female gender (OR 12.25 95% CI 2.37-63.24), reduced pulmonary function, (OR 5.11 95% CI 1.09-23.81) were each independent risk factors for mortality in CF.
  
  
  CONCLUSIONS
  Liver disease is an independent risk factor for mortality in CF.
authors.affiliation: School of Medicine and Medical Science University College Dublin, Catherine McAuley Research Centre, Nelson Street, Dublin 7, Ireland. Electronic address: marion.rowland@ucd.ie.
authors.forename: Marion
authors.initials: M
authors.lastname: Rowland
authors.affiliation: School of Medicine and Medical Science University College Dublin, Catherine McAuley Research Centre, Nelson Street, Dublin 7, Ireland.
authors.forename: Cliona
authors.initials: C
authors.lastname: Gallagher
authors.affiliation: St Vincent's University Hospital Elm Park, Dublin 4, Ireland; School of Medicine and Medical Science, Health Science Centre, Belfield, Dublin 4, Ireland.
authors.forename: Charles G
authors.initials: CG
authors.lastname: Gallagher
authors.affiliation: St Vincent's University Hospital Elm Park, Dublin 4, Ireland; School of Medicine and Medical Science, Health Science Centre, Belfield, Dublin 4, Ireland.
authors.forename: Risteárd Ó
authors.initials: RÓ
authors.lastname: Laoide
authors.affiliation: Our Lady's Children's Hospital Crumlin, Dublin 12, Ireland.
authors.forename: Gerard
authors.initials: G
authors.lastname: Canny
authors.affiliation: School of Medicine and Medical Science, Health Science Centre, Belfield, Dublin 4, Ireland; Our Lady's Children's Hospital Crumlin, Dublin 12, Ireland.
authors.forename: Anne Marie
authors.initials: AM
authors.lastname: Broderick
authors.affiliation: School of Medicine and Medical Science University College Dublin, Catherine McAuley Research Centre, Nelson Street, Dublin 7, Ireland.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Drummond
authors.affiliation: The National Children's Hospital, Tallaght, Dublin 14, Ireland.
authors.forename: Peter
authors.initials: P
authors.lastname: Greally
authors.affiliation: St Vincent's University Hospital Elm Park, Dublin 4, Ireland; The Children's University Hospital, Temple Street, Dublin 7, Ireland.
authors.forename: Dubhfeasa
authors.initials: D
authors.lastname: Slattery
authors.affiliation: UCD School of Public Health, Physiotherapy, and Population Science, Dublin 4, Ireland.
authors.forename: Leslie
authors.initials: L
authors.lastname: Daly
authors.affiliation: Beaumont Hospital, Dublin 9, Ireland; Royal College of Surgeons in Ireland, Dublin 2, Ireland.
authors.forename: Noel G
authors.initials: NG
authors.lastname: McElvaney
authors.affiliation: St Vincent's University Hospital Elm Park, Dublin 4, Ireland; Our Lady's Children's Hospital Crumlin, Dublin 12, Ireland.
authors.forename: Billy
authors.initials: B
authors.lastname: Bourke
mesh_terms: D000293:Adolescent; D017677:Age Distribution; D016022:Case-Control Studies; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D016001:Confidence Intervals; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007494:Ireland; D008107:Liver Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods / Q000401:mortality; D016015:Logistic Models; D016040:Lung Transplantation / Q000379:methods / Q000401:mortality; D008297:Male; D015999:Multivariate Analysis; D016017:Odds Ratio; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D016019:Survival Analysis; D016896:Treatment Outcome
keywords: Cystic fibrosis liver disease; Female gender; Mortality; Nutrition; Outcome