Literature DB >> 24917116

Outcome in patients with cystic fibrosis liver disease.

Marion Rowland1, Cliona Gallagher2, Charles G Gallagher3, Risteárd Ó Laoide3, Gerard Canny4, Anne Marie Broderick5, Jennifer Drummond2, Peter Greally6, Dubhfeasa Slattery7, Leslie Daly8, Noel G McElvaney9, Billy Bourke10.   

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.
Copyright © 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Entities:  

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

Mesh:

Year:  2014        PMID: 24917116     DOI: 10.1016/j.jcf.2014.05.013

Source DB:  PubMed          Journal:  J Cyst Fibros        ISSN: 1569-1993            Impact factor:   5.482


  16 in total

Review 1.  Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases.

Authors:  Luca Fabris; Romina Fiorotto; Carlo Spirli; Massimiliano Cadamuro; Valeria Mariotti; Maria J Perugorria; Jesus M Banales; Mario Strazzabosco
Journal:  Nat Rev Gastroenterol Hepatol       Date:  2019-08       Impact factor: 46.802

2.  Blocking follistatin-like 1 attenuates liver fibrosis in mice by regulating transforming growth factor-beta signaling.

Authors:  Xiao-Hua Zhang; Yong Chen; Bin Li; Ji-Yong Liu; Chong-Mei Yang; Ming-Ze Ma
Journal:  Int J Clin Exp Pathol       Date:  2018-03-01

3.  Genetic variation in CFTR and modifier loci may modulate cystic fibrosis disease severity.

Authors:  Alekh Paranjapye; Manon Ruffin; Ann Harris; Harriet Corvol
Journal:  J Cyst Fibros       Date:  2019-11-14       Impact factor: 5.482

Review 4.  Cystic fibrosis liver disease: A condition in need of structured transition and continuity of care.

Authors:  Julian Hercun; Fernando Alvarez; Catherine Vincent; Marc Bilodeau
Journal:  Can Liver J       Date:  2019-08-27

5.  Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis.

Authors:  Jaclyn R Stonebraker; Chee Y Ooi; Rhonda G Pace; Harriet Corvol; Michael R Knowles; Peter R Durie; Simon C Ling
Journal:  Clin Gastroenterol Hepatol       Date:  2016-04-05       Impact factor: 11.382

6.  Identification of growth differentiation factor 15 as a pro-fibrotic factor in mouse liver fibrosis progression.

Authors:  Peng Qi; Ming-Ze Ma; Jing-Hua Kuai
Journal:  Int J Exp Pathol       Date:  2021-05-13       Impact factor: 2.793

Review 7.  Epithelial Anion Transport as Modulator of Chemokine Signaling.

Authors:  Andrea Schnúr; Péter Hegyi; Simon Rousseau; Gergely L Lukacs; Guido Veit
Journal:  Mediators Inflamm       Date:  2016-06-12       Impact factor: 4.711

Review 8.  Imaging the Abdominal Manifestations of Cystic Fibrosis.

Authors:  C D Gillespie; M K O'Reilly; G N Allen; S McDermott; V O Chan; C A Ridge
Journal:  Int J Hepatol       Date:  2017-01-29

9.  Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients.

Authors:  Pierre-Yves Boëlle; Dominique Debray; Loic Guillot; Annick Clement; Harriet Corvol
Journal:  Hepatology       Date:  2018-12-28       Impact factor: 17.425

10.  Nutritional status of adolescents with cystic fibrosis treated at a reference center in the southeast region of Brazil.

Authors:  Ieda Regina Lopes Del Ciampo; Luiz Antonio Del Ciampo; Regina Sawamura; Laiane Renolfi de Oliveira; Maria Inez Machado Fernandes
Journal:  Ital J Pediatr       Date:  2015-07-30       Impact factor: 2.638

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