Closing in on Biliary Atresia

Winita      Hardikar; Kathleen   B.   Schwarz

Abstract

Biliary atresia (BA) is a rare congenital condition which results in significant morbidity and mortality. The Kasai hepatic portoenterostomy and liver transplantation have significantly improved survival in this disease; however, unraveling the etiology/etiologies will be required to improve transplantation free survival. At present, BA is still the single most common condition in children on pediatric liver transplant waiting lists and accounts for up to 50% of those waiting for liver transplantation [1]. It is the most rapidly fibrosing liver disease in man and accounts for ∼10% of all liver transplant procedures performed [2]. The rarity of this condition and the likelihood of a multi-factorial etiology have limited progress in research. Recently, collaborative research networks in North America (BARC) and Europe (EBAR) have been established [3,4]. The following discussion outlines the most recent information available from these and other groups concerning epidemiology, pathophysiology, etiology, diagnosis, management, long term outcome and conclusions/ recommendations for future research.

Keywords: Biliary atresia, neonatal cholestasis, cholangiopathy, hepatic fibrosis, portoenterostomy, biliary epithelial cells

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