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儿童肝移植:长期疗效与生活质量

Liver transplantation in children: long-term outcome and quality of life.

作者信息

Burdelski M, Nolkemper D, Ganschow R, Sturm E, Malago M, Rogiers X, Brölsch C E

机构信息

Kinderklinik, Universitätskrankenhaus Eppendorf, Martinistr. 52, D-20246 Hamburg.

出版信息

Eur J Pediatr. 1999 Dec;158 Suppl 2:S34-42. doi: 10.1007/pl00014322.

Abstract

UNLABELLED

Liver transplantation has become a standard therapy in acute and chronic liver failure. Since 1968, 2554 paediatric patients receiving a liver transplant have been registered in the European Liver Transplant Registry (ELTR). Compared with 22,600 total transplants registered in the ELTR over the same period of time this means that about 10% of all liver transplants performed in Europe concern paediatric recipients, aged from 0 to 15 years. The indications in the paediatric population differ significantly from those of adult patients: More than 50% of patients suffer from cholestatic disorders, followed by hepatic based metabolic disorders, acute liver failure, non-cholestatic cirrhosis and liver tumours. The results of liver transplantation in paediatric patients have improved remarkably since the early 1980s. In 1997 a survival rate of 80% is almost the international standard. This improvement is due to the use of better immunosuppressive agents such as cyclosporin A and tacrolimus, followed by improvement in surgical techniques and finally by improvement in intensive care, better diagnostic tools for viral, bacterial and fungal infections and corresponding appropriate therapies. Quality of life as a measure of transplant results has not been sufficiently studied. The majority of paediatric liver transplant recipients has a good quality of life; only 10% suffer from significant morbidity. The impact of pretransplant damage to other organs such as brain, kidneys, bone and lungs and the influence of immunosuppression on somatic growth, neurological development, infection and metabolic balance are subjects of increasing concern.

CONCLUSION

The results available today show convincing evidence that liver transplantation is a therapeutic option in otherwise fatal hepatic disorders. Much effort, however, has to be made in order to achieve further improvements by increasing our knowledge of the pathophysiology of both pre- and posttransplant conditions.

摘要

未标注

肝移植已成为急性和慢性肝衰竭的标准治疗方法。自1968年以来,欧洲肝移植登记处(ELTR)已登记了2554例接受肝移植的儿科患者。与同期ELTR登记的22600例肝移植总数相比,这意味着在欧洲进行的所有肝移植中,约10%是针对0至15岁的儿科受者。儿科患者的适应症与成人患者有显著差异:超过50%的患者患有胆汁淤积性疾病,其次是肝脏代谢性疾病、急性肝衰竭、非胆汁淤积性肝硬化和肝肿瘤。自20世纪80年代初以来,儿科患者肝移植的结果有了显著改善。1997年,80%的生存率几乎成为国际标准。这种改善归因于使用了更好的免疫抑制剂,如环孢素A和他克莫司,其次是手术技术的改进,最后是重症监护的改善、更好的病毒、细菌和真菌感染诊断工具以及相应的适当治疗。作为移植结果衡量指标的生活质量尚未得到充分研究。大多数儿科肝移植受者生活质量良好;只有10%的患者有严重的发病率。移植前对其他器官如脑、肾、骨和肺的损害以及免疫抑制对身体生长、神经发育、感染和代谢平衡的影响越来越受到关注。

结论

目前可得的结果显示出令人信服的证据,表明肝移植是治疗其他致命性肝脏疾病的一种治疗选择。然而,为了通过增加我们对移植前后病理生理学的了解来实现进一步改善,还必须做出很大努力。

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