皮质类固醇
皮质类固醇有抗炎、免疫调节特性,可增加非胆盐依赖性胆汁流动。尽管存在这些可能的机制,但皮质类固醇疗法的好处尚未得到证明,已知的益处不足以抵消使用大剂量皮质类固醇所带来的相关风险。争论焦点集中在几个问题上,包括风险获益比、最佳剂量、最佳逐渐减量方案以及短期-长期获益。[69]Petersen C, Harder D, Melter M, et al. Postoperative high-dose steroids do not improve mid-term survival with native liver in biliary atresia. Am J Gastroenterol. 2008;103:712-719.http://www.ncbi.nlm.nih.gov/pubmed/18177447?tool=bestpractice.com[70]Meyers RL, Book LS, O'Gorman MA, et al. High-dose steroids, ursodeoxycholic acid, and chronic intravenous antibiotics improve bile flow after Kasai procedure in infants with biliary atresia. J Pediatr Surg. 2003;38:406-411.http://www.ncbi.nlm.nih.gov/pubmed/12632357?tool=bestpractice.com[71]Escobar MA, Jay CL, Brooks RM, et al. Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy. J Pediatr Surg. 2006;41:99-103.http://www.ncbi.nlm.nih.gov/pubmed/16410116?tool=bestpractice.com[72]Dillon PW, Owings E, Cilley R, et al. Immunosuppression as adjuvant therapy for biliary atresia. J Pediatr Surg. 2001;36:80-85.http://www.ncbi.nlm.nih.gov/pubmed/11150442?tool=bestpractice.com[73]Davenport M, Stringer MD, Tizzard SA, et al. Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia. Hepatology. 2007;46:1821-1827.http://onlinelibrary.wiley.com/doi/10.1002/hep.21873/fullhttp://www.ncbi.nlm.nih.gov/pubmed/17935230?tool=bestpractice.com[74]Davenport M, Parsons C, Tizzard S, et al. Steroids in biliary atresia: single surgeon, single centre, prospective study. J Hepatol. 2013;59:1054-1058.http://www.ncbi.nlm.nih.gov/pubmed/23811305?tool=bestpractice.com[75]Nio M, Muraji T; Japanese Biliary Atresia Society. Multicenter randomized trial of postoperative corticosteroid therapy for biliary atresia. Pediatr Surg Int. 2013;29:1091-1095.http://www.ncbi.nlm.nih.gov/pubmed/23989522?tool=bestpractice.com一项随机安慰剂对照临床试验以 140 名胆道闭锁婴儿为研究对象,研究皮质类固醇的治疗效果。静脉使用甲基泼尼松龙后给予口服泼尼松龙,共治疗 13 周,肝门肠吻合术术后 (post-HPE) 6 个月、24 个月时评估结果。与安慰剂组相比,皮质类固醇治疗组的患儿在 HPE 术后 6 个月未观察到具有统计学意义的胆汁引流获益。此外,治疗组 2 岁时自体肝生存率并无统计学意义上的显著改善。皮质类固醇治疗组的患儿不良事件发作时间比安慰剂组患儿要早,这一差异具有统计学意义。研究结果不支持常规使用皮质类固醇治疗胆道闭锁患儿。[76]Bezerra JA, Spino C, Magee JC, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA. 2014;311:1750-1759.http://www.ncbi.nlm.nih.gov/pubmed/24794368?tool=bestpractice.com