The main problem with biliary atresia (BA) is three-fold. Early diagnosis and timely therapy is mandatory in this rare and life-threatening disease. In as much as the aetiology of BA is unknown, all therapeutic attempts are still symptomatically orientated and finally, at the end-stage of the disease, the majority of the patients need organ replacement. Due to promising interdisciplinary cooperation and improved outcomes after liver transplantation, the overall survival rate of patients with BA increased remarkably during the last decades. Additionally, every effort was made in clinical and basic research to obtain a better understanding of the disease and its clinical course. Nevertheless, the nature of biliary atresia still remains unclear and therapeutic options are unsatisfactory. Numerous papers about BA have appeared, reflecting clinical and scientific activity. Considering recent publications and prospective activities, in the following we will summarise what is new in biliary atresia.
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