Interventions for preventing and managing advanced liver disease in cystic fibrosis
- 30 March 2020
- journal article
- review article
- Published by Wiley in Cochrane Database of Systematic Reviews
- Vol. 2020 (3), CD012056
- https://doi.org/10.1002/14651858.cd012056.pub3
Abstract
Background Cystic fibrosis is an autosomal recessive inherited defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene resulting in abnormal regulation of salt and water movement across the membranes. In the liver this leads to focal biliary fibrosis resulting in progressive portal hypertension and end‐stage liver disease in some individuals. This can be asymptomatic, but may lead to splenomegaly and hypersplenism, development of varices and variceal bleeding, and ascites; it has negative impact on overall nutritional status and respiratory function in this population. Prognosis is poor once significant portal hypertension is established. The role and outcome of various interventions for managing advanced liver disease (non‐malignant end stage disease) in people with cystic fibrosis is currently unidentified. This is an updated version of a previously published review. Objectives To review and assess the efficacy of currently available treatment options for preventing and managing advanced liver disease in children and adults with cystic fibrosis. Search methods We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. Date of last search: 19 November 2019. We also searched the reference lists of relevant articles and reviews and online trials registries. Date of last search: 01 January 2020. Selection criteria Any published and unpublished randomised controlled trials and quasi‐randomised controlled trials of advanced liver disease in cystic fibrosis with cirrhosis or liver failure, portal hypertension or variceal bleeding (or both). Data collection and analysis Authors independently examined titles and abstracts to identify potentially relevant trials, but none were eligible for inclusion in this review. Main results A comprehensive search of the literature did not identify any published eligible randomised controlled trials. Authors' conclusions In order to develop the best source of evidence, there is a need to undertake randomised controlled trials of interventions for preventing and managing advanced liver disease in adults and children with cystic fibrosis.Keywords
This publication has 80 references indexed in Scilit:
- Management of portal hypertension in childrenWorld Journal of Gastroenterology, 2012
- Treatment of portal hypertensionWorld Journal of Gastroenterology, 2012
- Management of Portal Hypertension in ChildrenCurrent Gastroenterology Reports, 2010
- Determination of the Minimal Clinically Important Difference Scores for the Cystic Fibrosis Questionnaire-Revised Respiratory Symptom Scale in Two Populations of Patients With Cystic Fibrosis and Chronic Pseudomonas aeruginosa Airway InfectionSocial psychiatry. Sozialpsychiatrie. Psychiatrie sociale, 2009
- Evolving Consensus in Portal Hypertension Report of the Baveno IV Consensus Workshop on methodology of diagnosis and therapy in portal hypertensionJournal of Hepatology, 2005
- Epidemiology of liver disease in cystic fibrosis: a longitudinal studyJournal of Hepatology, 2004
- Ursodeoxycholic acid improves the hepatic metabolism of essential fatty acids and retinol in children with cystic fibrosisThe Journal of Pediatrics, 1997
- Clinical denouement and mutation analysis of patients with cystic fibrosis undergoing liver transplantation for biliary cirrhosisThe Journal of Pediatrics, 1995
- Taurine supplementation, fat absorption, and growth in cystic fibrosisThe Journal of Pediatrics, 1987
- Propranolol therapy for portal hypertension in childrenThe Journal of Pediatrics, 1985