Cystic fibrosis related liver disease--another black box in hepatology

Int J Mol Sci. 2014 Aug 4;15(8):13529-49. doi: 10.3390/ijms150813529.

Abstract

Due to improved medical care, life expectancy in patients with cystic fibrosis (CF) has veritably improved over the last decades. Importantly, cystic fibrosis related liver disease (CFLD) has become one of the leading causes of morbidity and mortality in CF patients. However, CFLD might be largely underdiagnosed and diagnostic criteria need to be refined. The underlying pathomechanisms are largely unknown, and treatment strategies with proven efficacy are lacking. This review focuses on current invasive and non-invasive diagnostic standards, the current knowledge on the pathophysiology of CFLD, treatment strategies, and possible future developments.

Publication types

  • Review

MeSH terms

  • Biomarkers / blood
  • Carrier Proteins / metabolism
  • Cholangitis, Sclerosing / diagnosis
  • Cholangitis, Sclerosing / etiology
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / metabolism
  • Diagnosis, Differential
  • Humans
  • Liver Diseases / diagnosis*
  • Liver Diseases / etiology
  • Liver Diseases / therapy
  • Liver Transplantation
  • Membrane Glycoproteins / metabolism
  • Non-alcoholic Fatty Liver Disease / diagnosis
  • Non-alcoholic Fatty Liver Disease / etiology

Substances

  • Biomarkers
  • Carrier Proteins
  • Membrane Glycoproteins
  • bile acid binding proteins