After Two Centuries Biliary Atresia Remains the Darkest Chapter in Pediatric Hepatology - Abstract
Two centuries after Professor John Burns, of the Department of Surgery, University of Glasgow referred to biliary atresia in his text book the etiology of the obliterative process remains a mystery. We still lack a complete understanding of the pathogenesis of the disorder, which hurdled our efforts to develop preventive and therapeutic measures. Several genetic, immune, viral, toxic and vascular etiologies have been proposed without conclusive evidence. Early recognition and prompt intervention are imperative in order to prevent rapid progressive damage to the liver. Although liver transplantation has been proposed as a primary therapy, the Kasai procedure (hepatoportoenterostomy remains the most reasonable initial approach. Biliary atresia is the most common indication for liver transplantation in the pediatric age group accounting for at least 50% of all transplants.