Strengthening collaboration within Europe: biliary atresia and the rise of the European Reference Networks.

Abstract

Biliary atresia (BA) is a rare and devastating cholangiopathy with an incidence of 1:15 000-20 000 in Europe. There is a consensus that BA and related rare diseases should be managed at centers of expertise. However, current BA care in Europe is heterogeneous, with decentralized treatment in the majority of European countries. The varying outcomes in Europe, referring to overall and native liver survival following Kasai procedure, have led to an ongoing discussion on international clinical and scientific collaborations. In 2017, the European Reference Networks (ERN) have been launched by the European Commission to improve the treatment of rare diseases. The ERNs bring together centers of expertise, which undergo qualification and monitoring processes. The European Reference Network on Rare Hepatological Diseases (ERN RARE-LIVER) represents a network of medical practitioners and patient representatives with the goal of improving care for rare liver diseases with BA as one key disorder. Exchange programs, digital case discussions and the endorsement of clinical studies are part of the RARE-LIVER agenda, including the prospective European Biliary Atresia Registry (EBAR), which has recently been launched. Such sustainable joint European efforts and strategies are crucial to improve BA outcomes in the short term and long term.