Efficacy of New Post Kasai ILBS Protocol in Biliary Atresia.

Biliary atresia (BA) is a neonatal progressive fibrosing cholan- giopathy and the most frequent indication for pediatric liver trans- plantation [1]. Surgical removal of biliary remnants and Roux-en-Y hepatoportoenterostomy (HPE) aims to restore biliary drainage and suppress progression to cirrhosis. Successful HPE, defined as a serum total bilirubin level <2 mg/dL at three months after surgery, occurs in ∼50% of patients in the United States [2]. Young age seems to be the best predictor of response to HPE, with limited data on the efficacy of adjuvant therapies such as corticosteroids, antibiotics, and choleretic agents [3,4]. Potential modes of action of these therapies are to increase bile flow as well as exert an anti- inflammatory effect [5].

In 2007, a double-blind randomized trial in the United Kingdom identified a beneficial effect on corticosteroid therapy on reduction of bilirubin level at one month post HPE without sig- nificant change in the need for liver transplantation [6]. Since then there have been multiple trial most prominent being, Kings hospital trial [7] and START trial [8] which demonstrated reduction in bilirubin levels; however both failed to demonstrate any effect on native liver survival.

However one study done by Bezerra et al [9] where they employed steroid in customised manner showed significant improvement in bile drainage in their subjects versus their historical cohort. Hence we propose to perform a prospective cohort study to assess the Efficacy of new post Kasai (steroid) ILBS protocol in Biliary Atresia.