Imperial College London

Shahid A Khan

Faculty of MedicineFaculty of Medicine Centre

Professor of Practice (Haematology)



+44 (0)20 3312 6254shahid.khan




Queen Elizabeth the Queen Mother Wing (QEQM)St Mary's Campus






BibTex format

author = {Toledano, M and Mukherjee, S and Howell, J and Westaby, D and Khan, S and Bilton, D and Simmonds, N},
doi = {10.1371/journal.pone.0212779},
journal = {PLoS ONE},
title = {The emerging burden of liver disease in cystic fibrosis patients: a UK nationwide study},
url = {},
volume = {14},
year = {2019}

RIS format (EndNote, RefMan)

AB - 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.
AU - Toledano,M
AU - Mukherjee,S
AU - Howell,J
AU - Westaby,D
AU - Khan,S
AU - Bilton,D
AU - Simmonds,N
DO - 10.1371/journal.pone.0212779
PY - 2019///
SN - 1932-6203
TI - The emerging burden of liver disease in cystic fibrosis patients: a UK nationwide study
UR -
UR -
VL - 14
ER -