肝移植治疗威尔森氏病:当前知识与未来展望。

IF 4.7 2区 医学 Q1 GASTROENTEROLOGY & HEPATOLOGY
Liver Transplantation Pub Date : 2024-12-01 Epub Date: 2024-06-19 DOI:10.1097/LVT.0000000000000422
Alberto Ferrarese, Nora Cazzagon, Patrizia Burra
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引用次数: 0

摘要

目前,对于出现终末期肝病或急性肝功能衰竭的威尔逊氏病患者来说,肝移植是一种治疗选择。事实上,由于移植时年龄较小,且无复发,因此术后生存曲线非常好。过去几十年来,人们的注意力已转移到明智地扩大肝移植的适应症上。有证据表明,经过精心挑选、主要伴有神经精神症状和代偿性肝硬化的患者可以接受移植手术。采用这种方法的理由是,手术有可能改善铜平衡,从而改善神经精神症状。然而,仍有几个问题尚未解决,例如如何确定移植前神经精神损伤的评估阈值、如何规范术前神经评估以及如何定义符合这些特定标准的患者的术后结果。此外,我们还将针对威尔逊氏病患者的肝移植评估和移植等待期间的患者护理,提出一种针对特定疾病的方法,强调这种全身性疾病的特殊性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Liver transplantation for Wilson disease: Current knowledge and future perspectives.

Liver transplantation currently represents a therapeutic option for patients with Wilson disease presenting with end-stage liver disease or acute liver failure. Indeed, it has been associated with excellent postoperative survival curves in view of young age at transplant and absence of recurrence. Attention has shifted over the past decades to a wise expansion of indications for liver transplantation. Evidence has emerged supporting the transplantation of carefully selected patients with primarily neuropsychiatric symptoms and compensated cirrhosis. The rationale behind this approach is the potential for surgery to improve copper homeostasis and consequently ameliorate neuropsychiatric symptoms. However, several questions remain unanswered, such as how to establish thresholds for assessing pretransplant neuropsychiatric impairment, how to standardize preoperative neurological assessments, and how to define postoperative outcomes for patients meeting these specific criteria. Furthermore, a disease-specific approach will be proposed both for the liver transplant evaluation of candidates with Wilson disease and for patient care during the transplant waiting period, highlighting the peculiarities of this systemic disease.

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来源期刊
Liver Transplantation
Liver Transplantation 医学-外科
CiteScore
7.40
自引率
6.50%
发文量
254
审稿时长
3-8 weeks
期刊介绍: Since the first application of liver transplantation in a clinical situation was reported more than twenty years ago, there has been a great deal of growth in this field and more is anticipated. As an official publication of the AASLD, Liver Transplantation delivers current, peer-reviewed articles on liver transplantation, liver surgery, and chronic liver disease — the information necessary to keep abreast of this evolving specialty.
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