Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy

Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy
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DOI:
10.1016/j.jpedsurg.2005.10.072
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发表时间:
2006-01-01
影响因子:
2.4
通讯作者:
Grosfeld, JL
Grosfeld, JL
中科院分区:
医学3区
文献类型:
--
作者:
Escobar, MA;Jay, CL;Grosfeld, JL

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目的:本研究测试的假设,类固醇管理改善胆道闭锁(BA)的结果,通过评估术后类固醇使用的疗效与BA的婴儿手术结果的假设。方法:类固醇的使用和BA患者的结果进行了回顾性分析,在三级儿科医院。结果:加塞门肠吻合术(PE)进行了43例BA治疗1992年至2004年(16名男孩和27名女孩)。21例PE患者接受类固醇治疗,22例未接受。24例(55.8%)血清胆红素持续低于2 mg/dL的患者成功进行了门肠吻合术。16例(66%)接受术后类固醇。术后6个月时,21例使用类固醇的患者中有16例(76%)胆红素正常,而未接受治疗的对照组中有8例(37%)胆红素正常(Fisher精确检验,P = 0.01)。在43例患者中,19例(44%)需要肝移植,其中7例(37%)使用类固醇,12例(63%)未使用类固醇(P = .2)。28名婴儿发生胆管炎(发热伴或不伴肝功能变化):PE后25名,移植后3名。在25例患者中,12例(48%)接受了类固醇治疗。7例死亡(16%)(范围:7个月至4年):2例在等待移植期间(接受类固醇),5例在移植后(1例接受类固醇,4例未接受治疗)。使用类固醇的患者生存率为86%(18/21),未使用类固醇的患者生存率为82%(18/22)。移植存活率(741/6)与以前报道的历史对照(82%)。结论:加塞PE仍然是婴儿BA小于3个月的选择程序。在接受类固醇的患者中观察到术后黄疸的清除率显著提高,血清胆红素水平降低。然而,类固醇对胆管炎的发生率、肝移植的需要和总生存率没有影响。一项前瞻性研究,标准化剂量和长度的类固醇管理和更长的随访时间是必要的,以更准确地评估类固醇的有效性后PE。(c)2006年爱思唯尔公司All rights reserved.
Purpose: This study tests the hypothesis that steroid administration improves the outcome of biliary atresia (BA) by evaluating the efficacy of postoperative steroid use on surgical outcomes in infants with BA.Methods: Steroid use and outcomes in patients with BA were retrospectively analyzed at a tertiary pediatric hospital. Institutional review board approval was obtained.Results: Kasai portoenterostomy (PE) was performed in 43 patients with BA treated from 1992 to 2004 (16 boys and 27 girls). Twenty-one PE patients received steroids and 22 did not. Portoenterostomy was successful in 24 patients (55.8%) with consistent serum bilirubin less than 2 mg/dL. Sixteen (66%) received postoperative steroids. A normal postoperative bilirubin was achieved at 6 months in 16 (76%) of 21 patients with steroids compared with 8 (37%) of 22 in untreated controls (Fisher's Exact test, P = .01). Of the 43 patients, 19 (44%) required liver transplantation, including 7 (37%) of 19 with steroids vs 12 (63%) of 19 without (P = .2). Twenty-eight infants developed cholangitis (fever with and without changes in hepatic function): 25 after PE and 3 after transplant. Of the 25, 12 (48%) received steroids. Seven died (16%) (range, 7 months to 4 years): 2 while awaiting transplantation (received steroids) and 5 after transplantation (1 received steroids and 4 were untreated). Survival was 86% (18/21) in patients with steroids and 82% (18/22) in those without. Transplant survival (741/6) was comparable to previously reported historical controls (82%).Conclusions: The Kasai PE continues to be the procedure of choice in infants with BA younger than 3 months. A significantly improved clearance of postoperative jaundice and lower serum bilirubin levels were observed in patients receiving steroids. However, steroids had no effect on the incidence of cholangitis, need for liver transplantation, and overall survival. A prospective study with standardized dose and length of steroid administration and longer period of follow-up is necessary to more accurately assess the effectiveness of steroids after PE. (c) 2006 Elsevier Inc. All rights reserved.