Journal article

The emerging burden of liver disease in cystic fibrosis patients: A UK nationwide study

MB Toledano, SK Mukherjee, J Howell, D Westaby, SA Khan, D Bilton, NJ Simmonds



OBJECTIVE: Cystic 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. METHODS: A 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. RESULTS: Prevalence of CFLD increased from 203.4 to 228.3 per 1000 patients during 2008-2013. Mortal..

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Funding Acknowledgements

S K Mukherjee is supported by NIHR Academic Clinical Fellowship. J Howell is supported by an NHMRC (Australia) Early Career Fellowship. D Bilton and NJ Simmonds are supported by the NIHR funded specialist Respiratory Biomedical Research Unit at Royal Brompton and Harefield NHS Foundation Trust and Imperial College. NJ Simmonds has consulted for Vertex, Gilead, Chiesi, Teva, Roche and Pulmocide. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.