Effect of Adjuvant Steroid Therapy in Type 3 Biliary Atresia

Abstract

Objective: To evaluate the efficacy and side effects of additional postoperative steroid therapy for type 3 BA versus the current routine care. Summary Background Data: Whether steroid therapy post-Kasai portoen-terostomy improves the outcomes of BA remains controversial. Clinical evidence from 2 randomized trials in the UK and USA do not support the routine use of steroid in the treatment of BA. Methods: In this open-label randomized controlled trial, patients with type 3 BA were randomized to routine postoperative treatment with or without 10 to 12 weeks of adjuvant steroid treatment. The primary outcome was the postoperative jaundice clearance rate with native liver at 6 months. The secondary outcomes included postoperative jaundice clearance rate at 3, 12, and 24 months, survival with native liver at 12 and 24 months, and SAEs within 3 months. Results: Overall, 200 participants were randomized and allocated into either steroid or control group (n = 100/group). The proportion of participants that are jaundice free without liver transplantation was significantly higher in the steroid group than in the control group at 6 months (54.1% vs 31.0%, P = 0.0015). The native liver survival rate was higher postoperatively in the steroid group than in the control group at 12 (66.3% vs 50.0%, P = 0.02) and 24 (57.1% vs 40.0%, P = 0.02) months. The survival time with native liver was significantly longer in the steroid group than in the control group (median survival, steroid vs control: not reached vs 1.21 years, P = 0.02). There were no significant differences between the 2 groups in the mean occurrence of SAEs within 3 months (steroid vs control: 0.63 vs 0.45, P = 0.20). Conclusions: Postoperative adjuvant steroid intervention improved bile drainage and survival with native liver in type 3 BA patients, without increasing early-stage SAEs.

Steroid therapy in extrahepatic cholestasis suggestive of biliary atresia: A case report

“…Bilirubin levels every two weeks of therapy 4842 atresia patients after KPE may provide a better result than those who are not administered. 17,18 However, there are no studies regarding the efficacy of administering steroids before KPE, especially when cholestasis develops in young infants.…”

Major surgical conditions of childhood and their lifelong implications: comprehensive review

“…After surgery, the use of steroids is widely adopted for its anti-inflammatory effect and promotion of bile flow. A recent RCT and meta-analysis both confirmed that postoperative adjuvant steroid therapy improved bile drainage and native liver survival 161 , 162 . Recurrent cholangitis is another significant factor which negatively affects native liver survival.…”