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.
See this in plain English?
AI-rewrites the medical criteria so a patient or caregiver can understand them. Always confirm with the trial site.
We expect steroids used in customized manner to improve biliary drainage and thereby improve native liver survival in post kasai patients.
Timeframe: Within 6 months