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Plasma exchange for hemolytic crisis and acute liver failure in Wilson disease: Authors' reply

机译:血浆置换治疗威尔逊病中的溶血危机和急性肝衰竭:作者的回复

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To the Editor. We thank Dr Kumar for taking interest in our article [1]. We agree with the author that use of modalities like plasmapheresis should be considered in Wilson's disease- Acute liver failure (WD-ALF); as seen in our case and in other cases mentioned by the author, plasmapheresis can either provide a bridge to liver transplant or rarely prevent the transplant. The commonly used prognostic scoring system- the revised Wilson's Prognostic Index was devised after retrospective review of 74 Wilsons disease (WD) children at the King's College Hospital, London between 1967 and 2000 [2]. The revised score was tested prospec-tively in 14 WD children between 2001 and 2003 [2]. The validity of this score in the present era with availability of newer hepatic assist systems remains to be established in a larger number of patients. Over-reliance on the prognostic scoring systems may result in unnecessary transplants and misallocation of a rare resource [3].
机译:致编辑。我们感谢Kumar博士对我们的文章[1]感兴趣。我们同意作者的观点,在威尔逊氏病-急性肝衰竭(WD-ALF)中应考虑使用血浆置换等方法。从我们的案例以及作者提到的其他案例中可以看出,血浆置换可以为肝移植提供桥梁,也很少阻止肝移植。 1967年至2000年间,在伦敦国王学院医院对74名威尔逊氏病(WD)儿童进行回顾性研究后,设计了常用的预后评分系统-修订后的威尔逊预后指数[2]。在2001年至2003年之间,对14名WD儿童进行了修订后的评分测试[2]。在当今时代,随着新型肝辅助系统的可用性,该评分的有效性仍需在更多患者中建立。过度依赖预后评分系统可能会导致不必要的移植和稀有资源的错误分配[3]。

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