Outcome in patients with cystic fibrosis liver disease

J Cyst Fibros. 2015 Jan;14(1):120-6. doi: 10.1016/j.jcf.2014.05.013. Epub 2014 Jun 7.

Abstract

Background: Liver disease is an important complication in CF.

Aims: 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.

Keywords: Cystic fibrosis liver disease; Female gender; Mortality; Nutrition; Outcome.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Age Distribution
  • Case-Control Studies
  • Cause of Death*
  • Child
  • Child, Preschool
  • Comorbidity
  • Confidence Intervals
  • Cystic Fibrosis / diagnosis
  • Cystic Fibrosis / epidemiology*
  • Cystic Fibrosis / surgery*
  • Female
  • Follow-Up Studies
  • Humans
  • Incidence
  • Ireland
  • Liver Diseases / diagnosis
  • Liver Diseases / epidemiology*
  • Liver Diseases / surgery*
  • Liver Transplantation / methods
  • Liver Transplantation / mortality
  • Logistic Models
  • Lung Transplantation / methods
  • Lung Transplantation / mortality
  • Male
  • Multivariate Analysis
  • Odds Ratio
  • Risk Assessment
  • Severity of Illness Index
  • Sex Distribution
  • Survival Analysis
  • Treatment Outcome