The gallbladder and biliary tract in cystic fibrosis

Abstract

Chronic liver disease is a major complication of cystic fibrosis. Its incidence and severity are variable, and diagnosis relies on a combination of clinical evaluation, biochemical testing, and radiologic assessment. Identifying patients who have early disease is critical, and the administration of ursodeoxycholic acid appears to be beneficial. The pathogenesis is incompletely understood, and factors that contribute to the variability in incidence and severity are unknown. Fortunately, only a small proportion of individuals progress to advanced liver disease; however, in this population, there is significant morbidity and impairment in quality of life. Liver transplantation can be performed successfully in patients with end-stage liver disease. Future treatments involve targeted gene therapy and activation of mutant forms of the cystic fibrosis transmembrane conductance regulator.