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小儿肝移植的适应证。来自海德堡小儿肝移植项目的数据。

Indications for pediatric liver transplantation. Data from the Heidelberg pediatric liver transplantation program.

作者信息

Engelmann G, Schmidt J, Oh J, Lenhartz H, Wenning D, Teufel U, Büchler M W, Hoffmann G F, Meyburg J

机构信息

University of Heidelberg, Department of Paediatrics, INF 150, D-69120 Heidelberg, Germany.

出版信息

Nephrol Dial Transplant. 2007 Sep;22 Suppl 8:viii23-viii28. doi: 10.1093/ndt/gfm649.

Abstract

Nowadays liver transplantation is an established treatment for children with end-stage liver disease with very good 1- and 5-year survival. This has been achieved through constant improvement of surgical techniques, new immunosuppressive drugs and clinical management. Indications for liver transplantation in infants and children include acute liver failure (ALF), chronic liver failure with pruritus, complications of cholestasis and failure to thrive. In young children, the most common liver disease leading to transplantation is biliary atresia. Biliary atresia accounts for at least 50 percent of all liver transplants in children and is characterized by the failure of the bile ducts to develop normally and drain bile from the liver. Several models to assess prognosis of liver disease have been developed. In acute liver failure leukocyte count, bilirubin, International Normalized Ratio (INR) and age have a strong correlation with outcome. In chronic liver failure, PELD (Pediatric end-stage liver disease) Score and the occurrence of complications of liver disease are important prognostic tools. Since the start of our own paediatric liver transplantation program at the University of Heidelberg in 2003, already 15 Children between 5 months and 14 years have been transplanted. Indications and outcome of these patients are reviewed in this paper.

摘要

如今,肝移植是治疗终末期肝病患儿的一种成熟疗法,其1年和5年生存率都很高。这是通过不断改进手术技术、新型免疫抑制药物和临床管理实现的。婴儿和儿童肝移植的适应证包括急性肝衰竭(ALF)、伴有瘙痒的慢性肝衰竭、胆汁淤积并发症以及发育不良。在幼儿中,导致移植的最常见肝病是胆道闭锁。胆道闭锁占儿童所有肝移植的至少50%,其特征是胆管发育异常,无法从肝脏引流胆汁。已经开发了几种评估肝病预后的模型。在急性肝衰竭中,白细胞计数、胆红素、国际标准化比值(INR)和年龄与预后密切相关。在慢性肝衰竭中,小儿终末期肝病(PELD)评分和肝病并发症的发生情况是重要的预后评估工具。自2003年我们在海德堡大学开展小儿肝移植项目以来,已有15名年龄在5个月至14岁之间的儿童接受了移植。本文对这些患者的适应证和治疗结果进行了回顾。

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