Hepatobiliary disease in patients with cystic fibrosis.

PURPOSE OF REVIEW: This review explores the recent advances in knowledge regarding hepatobiliary disease in patients with cystic fibrosis. RECENT
FINDINGS: Hepatobiliary abnormalities associated with cystic fibrosis are varied in nature and range from defects attributable to the underlying genetic defect to those related to systemic disease and malnutrition. Novel research into the underlying pathogenesis of cystic fibrosis liver disease and the primary role of cystic fibrosis transmembrane conductance regulator in biliary secretory epithelium is presented. This work has been fostered by the development of new animal models of cystic fibrosis transmembrane conductance regulator dysfunction. Of the wide range of hepatobiliary complications associated with cystic fibrosis the most clinically relevant problem is progression of focal biliary cirrhosis to multilobular cirrhosis with its attendant complications of portal hypertension and potentially end-stage liver disease. However, recent studies suggest that liver transplantation may not improve survival in patients with cystic fibrosis and significant portal hypertension.
SUMMARY: Hepatobiliary disease is a common finding in patients with cystic fibrosis; the pathogenesis is multifactorial in nature. As new therapeutic strategies emerge, life expectancy will continue to increase as will the impact of liver disease on quality of life and survival of patients with cystic fibrosis. This review will discuss novel insights into pathogenesis as well as diagnostic and management options.