Purpose of review
This review looks at what is currently known about liver disease in cystic fibrosis (CF) in light of the literature over the past year, and what the ongoing challenges are from a clinical and research perspective for the optimal management of cystic fibrosis liver disease
Patients with CF who develop clinically significant liver disease have a worse overall phenotype
, and whereas there is no definite evidence that they have a shorter life expectancy, longer follow-up is required to determine if liver disease is a risk factor for mortality in CF.
The development of the ferret and pig animal models of CF with multiorgan involvement is an important breakthrough which will enhance our understanding of the pathogenesis of CFLD, and with which it is hoped novel therapeutic targets for the treatment of CFLD will be identified.
Whereas there is still no effective treatment for liver disease in CF, recent developments of animal models
of CFLD will enhance our capacity to develop new therapeutic targets and reduce the impact of liver disease on mortality in CF.