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Biliary Atresia (BA) is an uncommon, progressive and idiopathic fibro-obliterative cholangiopathy. The typical clinical features should be identified prompt in order to realize a Kasai Hepatoportoenterostomy (HPE) for restore the bile flow. However, despite HPE the BA remains the most common indication for liver transplantation in children. The lasts studies have shown the importance of virus, immunity and other environmental substances in pathogenesis of BA that is important in order to look for new therapeutic and preventive strategies. This article is a systematic review of the literature about actual evidence in BA. Keywords: Biliary atresia; Neonatal jaundice; Kasai hepatoportoenterostomy; Liver transplantation

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