Outcome in patients with cystic fibrosis liver disease

Marion Rowland; Cliona Gallagher; Charles G Gallagher; Risteárd Ó Laoide; Gerard Canny; Anne Marie Broderick; Jennifer Drummond; Peter Greally; Dubhfeasa Slattery; Leslie Daly; Noel G McElvaney; Billy Bourke

doi:10.1016/j.jcf.2014.05.013

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.

Authors

Affiliations

¹ School of Medicine and Medical Science University College Dublin, Catherine McAuley Research Centre, Nelson Street, Dublin 7, Ireland. Electronic address: [email protected].
² School of Medicine and Medical Science University College Dublin, Catherine McAuley Research Centre, Nelson Street, Dublin 7, Ireland.
³ St Vincent's University Hospital Elm Park, Dublin 4, Ireland; School of Medicine and Medical Science, Health Science Centre, Belfield, Dublin 4, Ireland.
⁴ Our Lady's Children's Hospital Crumlin, Dublin 12, Ireland.
⁵ School of Medicine and Medical Science, Health Science Centre, Belfield, Dublin 4, Ireland; Our Lady's Children's Hospital Crumlin, Dublin 12, Ireland.
⁶ The National Children's Hospital, Tallaght, Dublin 14, Ireland.
⁷ St Vincent's University Hospital Elm Park, Dublin 4, Ireland; The Children's University Hospital, Temple Street, Dublin 7, Ireland.
⁸ UCD School of Public Health, Physiotherapy, and Population Science, Dublin 4, Ireland.
⁹ Beaumont Hospital, Dublin 9, Ireland; Royal College of Surgeons in Ireland, Dublin 2, Ireland.
¹⁰ St Vincent's University Hospital Elm Park, Dublin 4, Ireland; Our Lady's Children's Hospital Crumlin, Dublin 12, Ireland.

PMID: 24917116
DOI: 10.1016/j.jcf.2014.05.013

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