Molecular basis of cholestatic diseases of surgical interest

Cholestasis constitutes one of the most common and severe manifestations of acquired or inherited liver disease. When manifest in early infancy, it is often life-threatening and usually requires surgical management. In many cases, liver transplantation is the only effective therapy. Extensive knowledge about the molecular mechanisms underlying several pediatric cholestatic disorders has been gained in recent years from studies in both experimental models and clinical forms. In this review, we focus on recent contributions to the knowledge of molecular basis of main pediatric cholestatic disorders, such as biliary atresia, Alagille syndrome, and familial intrahepatic cholestasis. For some of them, putative targets of therapeutic interest, such as interferon-γ and Farnesoid X receptor, have been proposed.

Index words:  Biliary atresia , Alagille syndrome , JAG1 , Progressive familial intrahepatic cholestasis