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儿童肝移植的当前结果及不断发展的适应证

Current results and evolving indications for liver transplantation in children.

作者信息

Kelly D A

机构信息

Birmingham Children's Hospital NHS Trust, United Kingdom.

出版信息

J Pediatr Gastroenterol Nutr. 1998 Aug;27(2):214-21. doi: 10.1097/00005176-199808000-00017.

DOI:10.1097/00005176-199808000-00017
PMID:9702657
Abstract

Liver transplantation continues to be successful and effective treatment for acute and chronic liver failure, and many important lessons have been learned. The development of innovative operative techniques has much reduced the waiting list mortality rate and has extended transplantation to younger and sicker children and to those with functionally normal livers who may benefit from auxiliary liver transplantation. The incidence and range of postoperative complications have improved with increased medical and surgical expertise. As information on long-term outcome for liver transplantation is gained, it is clear that many children will benefit from early elective liver transplantation before the development of significant growth or psychosocial retardation. Early transplantation is also indicated in children with cirrhosis and intrapulmonary shunting or cystic fibrosis with moderate lung disease. During the same period, evolving medical therapy has altered the natural history, patient selection, and timing of transplantation in children with tyrosinaemia type I, primary bile acid disorders, neonatal haemochromatosis, and potentially, cystic fibrosis. It is now clear that children with significant multisystem disease, such as mitochondrial disorders or severe systemic oxalosis, are no longer suitable candidates for liver transplantation. The successful development of liver transplantation has brought good quality life to many children and their families. There are still many lessons to learn and there are future challenges such as the ever-increasing problems of donor scarcity and the search for potent but less toxic immunosuppressive agents.

摘要

肝移植仍然是治疗急慢性肝衰竭的成功且有效的方法,并且我们已经吸取了许多重要的经验教训。创新手术技术的发展大大降低了等待名单上的死亡率,并将肝移植扩展到更年幼、病情更重的儿童以及那些肝脏功能正常但可能从辅助性肝移植中获益的儿童。随着医疗和外科专业知识的增加,术后并发症的发生率和范围有所改善。随着获得肝移植长期结果的信息,很明显许多儿童将在出现明显生长或心理社会发育迟缓之前从早期择期肝移植中获益。对于患有肝硬化和肺内分流或患有中度肺部疾病的囊性纤维化的儿童,也建议进行早期移植。在同一时期,不断发展的药物治疗改变了I型酪氨酸血症、原发性胆汁酸紊乱、新生儿血色素沉着症以及可能的囊性纤维化患儿的自然病程、患者选择和移植时机。现在很清楚,患有严重多系统疾病(如线粒体疾病或严重全身性草酸osis)的儿童不再适合作为肝移植的候选者。肝移植的成功发展给许多儿童及其家庭带来了高质量的生活。仍有许多经验教训需要吸取,并且未来还面临着诸多挑战,比如供体短缺问题日益严重以及寻找强效但毒性较小的免疫抑制剂。

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