ArticlePloS one2019
The emerging burden of liver disease in cystic fibrosis patients: A UK nationwide study.
Article in PloS one, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05229640 (Relationship Between the Development of Impaired Glucose Tolerance, the Phenotype of CFLD, and the Risk of Liver Fibrosis), which is not on this map. Cited by 22 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Relationship Between the Development of Impaired Glucose Tolerance, the Phenotype of CFLD, and the Risk of Liver Fibrosis
Who cites it
22 citing papers in PubMed, 78 citations in OpenAlex.
- Contemporary outcomes of isolated liver and combined liver-lung transplantation for cystic fibrosis in children.Journal of pediatric gastroenterology and nutrition · 2026Article
- Decoding liver injury in cystic fibrosis: How to tell drug-induced liver injury from cystic fibrosis liver disease.World journal of gastroenterology · 2026Review
- Prevalence and Impact of Chronic Liver Disease in Adult Patients Admitted With Cystic Fibrosis.Cureus · 2026Article
- Evaluating the Diagnostic Potential of the FIB-4 Index for Cystic Fibrosis-Associated Liver Disease in Adults: A Comparison with Transient Elastography.Journal of clinical medicine · 2025Article
- Hepatocellular Carcinoma in Cystic Fibrosis Liver Disease.Gastro hep advances · 2025Article
- Genetic engineering drives the breakthrough of pig models in liver disease research.Liver research (Beijing, China) · 2024Review
- Combined Organ Transplantation in Patients with Advanced Liver Disease.Seminars in liver disease · 2024Review
- Liver magnetic resonance elastography and fat fraction in pediatric patients with cystic fibrosis versus healthy children.Pediatric radiology · 2024Article
- Improving detection of cystic fibrosis related liver disease using liver fibrosis assessment tools.Heliyon · 2023Article
- Cystic fibrosis-related mortality in the United States from 1999 to 2020: an observational analysis of time trends and disparities.Scientific reports · 2023Observational
- Non-cirrhotic portal hypertension (obliterative portal venopathy) is the predominant form of chronic liver disease in cystic fibrosis.Irish journal of medical science · 2023Article
- Future Comorbidities in an Aging Cystic Fibrosis Population.Life (Basel, Switzerland) · 2023Review
- The Gut-Liver Axis in Pediatric Liver Health and Disease.Microorganisms · 2023Review
- Modifier Factors of Cystic Fibrosis Phenotypes: A Focus on Modifier Genes.International journal of molecular sciences · 2022Review
- Cystic fibrosis related liver disease and endocrine considerations.Journal of clinical & translational endocrinology · 2022Article
- Early intrahepatic duct defects in a cystic fibrosis porcine model.Physiological reports · 2021Article
- Current Treatment Options for Cystic Fibrosis-Related Liver Disease.International journal of molecular sciences · 2020Review
- Early pathogenesis of cystic fibrosis gallbladder disease in a porcine model.Laboratory investigation; a journal of technical methods and pathology · 2020Article
- The changing demography of the cystic fibrosis population: forecasting future numbers of adults in the UK.Scientific reports · 2020Article
- The impact of liver disease on mortality in cystic fibrosis - a systematic review protocol.HRB open research · 2020Article
Corrections and comments
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Authors and funding
7 authors at 5 institutions in 2 countries.
Funding
Abstract
objectiveCystic fibrosis associated liver disease (CFLD) is the third largest cause of mortality in CF. Our aim was to define the burden of CFLD in the UK using national registry data and identify risk factors for progressive disease.
methodsA longitudinal population-based cohort study was conducted. Cases were defined as all patients with CFLD identified from the UK CF Registry, 2008-2013 (n = 3417). Denominator data were derived from the entire UK CF Registry. The burden of CFLD was characterised. Regression analysis was undertaken to identify risk factors for cirrhosis and progression.
resultsPrevalence of CFLD increased from 203.4 to 228.3 per 1000 patients during 2008-2013. Mortality in CF patients with CFLD was more than double those without; cirrhotic patients had higher all-cause mortality (HR 1.54, 95% CI 1.09 to 2.18, p = 0.015). Median recorded age of cirrhosis diagnosis was 19 (range 5-53) years. Male sex, Pseudomonas airway infection and CF related diabetes were independent risk factors for cirrhosis. Ursodeoxycholic acid use was associated with prolonged survival in patients without cirrhosis.
conclusionsThis study highlights an important changing disease burden of CFLD. The prevalence is slowly increasing and, importantly, the disease is not just being diagnosed in childhood. Although the role of ursodeoxycholic acid remains controversial, this study identified a positive association with survival.
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Registered trials
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