High-dose steroids, ursodeoxycholic acid, and chronic intravenous antibiotics improve bile flow after Kasai procedure in infants with biliary atresia

The bile salt export pump

“…Taken together, these results indicate that not only the bile acid sensor FXR is crucial for the control of Bsep expression, but more complex regulatory cascades involving for example steroid receptors need to be considered as well. This is supported by the observation that patients with biliary atresia improve their bile flow rate after surgery with high-dose steroid treatment [64].…”

Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia

“…12,13 Thereafter, small uncontrolled studies suggested a benefit when they were used postoperatively as adjuvant therapy. [2][3][4][5]14 Nonetheless, actual evidence for their efficacy has been difficult to obtain, and to date there has been no attempt to conduct a randomized, placebo-controlled clinical trial.…”

“…All were retrospective reviews, 2 used a single-standard steroid regimen, 2,3 1 had no control group, 2 and 2 compared the steroid group to contemporaneous results in the same institutions. 3,5 All suggested that steroids were advantageous in the clearance of jaundice, although 1 study found no difference in the need for liver transplantation. 5 The incidence of biliary atresia in Japan approaches twice that of the United Kingdom, 1,15 North America, and Western Europe, and it is worthwhile to explore the practice of the Japanese and their experience with adjuvant corticosteroids.…”

“…Postoperative steroids have been used empirically for many years, with a number of retrospective, uncontrolled reports suggesting a benefit. [2][3][4][5] However, because of its rarity, there has never been any attempt at a randomized, placebo-controlled trial to test their value.…”

Corticosteroid treatment in biliary atresia: Tonic or toast?

“…Thus, there is a reasonable scientific justification for the potential benefits of steroid therapy in BA. The clinical use of steroids in BA has exploded in recent years following the publication of a series of reports [14][15][16][17][18][19][20] attesting to better outcomes with routine post-HPE steroid therapy compared to a variety of historical or concurrent control groups. Each report used a different maximum dose of steroids (1-10 mg/kg/day), had its own unique dose tapering schedule, and was of variable treatment duration (generally 1-3 months).…”