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皮质类固醇疗法改善了胆道闭锁的预后。

The improved outlook for biliary atresia with corticosteroid therapy.

作者信息

Muraji T, Higashimoto Y

机构信息

Department of Surgery, Kobe Children's Hospital, Sumaku, Japan.

出版信息

J Pediatr Surg. 1997 Jul;32(7):1103-6; discussion 1106-7. doi: 10.1016/s0022-3468(97)90408-5.

DOI:10.1016/s0022-3468(97)90408-5
PMID:9247243
Abstract

Controversy exists regarding the efficacy of corticosteroids on bile flow after Kasai portoenterostomy in biliary atresia (BA). Fourteen patients who had BA and underwent Kasai portojejunostomy between November 1990 and March 1996 were subject of this study. Corticosteroid therapy ("blast" type) was used for inadequate bile drainage. Corticosteroid support was unnecessary in one patient with good bile drainage, and corticosteroids were aggressively used in the remaining 13 patients. Two patients who had no response to an initial blast subsequently responded and now are doing well. The remaining 11 patients responded to corticosteroids with varying degrees. Three had a limited response, and two ultimately underwent liver transplantation. There was one death caused by subdural hematoma. Three had an excellent initial response. However, one subsequently deteriorated because of intractable cholangitis, requiring liver transplantation. Ten survivors with native liver are anicteric with satisfactory growth and quality of life. Aggressive corticosteroid therapy is an important part of the management after Kasai portoenterostomy. The initial response to steroids does not necessarily reflect the final outcome.

摘要

关于皮质类固醇对胆道闭锁(BA)患者Kasai肝门空肠吻合术后胆汁流动的疗效存在争议。本研究的对象为1990年11月至1996年3月期间接受Kasai肝门空肠吻合术的14例BA患者。皮质类固醇疗法(“冲击”型)用于胆汁引流不足的情况。1例胆汁引流良好的患者无需皮质类固醇支持,其余13例患者积极使用了皮质类固醇。2例对初始冲击无反应的患者随后出现反应,目前情况良好。其余11例患者对皮质类固醇有不同程度的反应。3例反应有限,2例最终接受了肝移植。有1例因硬膜下血肿死亡。3例最初反应良好。然而,其中1例随后因难治性胆管炎病情恶化,需要进行肝移植。10例存活的自体肝患者无黄疸,生长情况及生活质量令人满意。积极的皮质类固醇疗法是Kasai肝门空肠吻合术后治疗的重要组成部分。对类固醇的初始反应不一定反映最终结果。

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