2025年4月，欧洲肝病学会发布了最新版肝豆状核变性指南，与2012年版肝豆状核变性指南相比，新版指南对疾病的临床特征、诊断流程、治疗策略、监测方法等方面均进行了更细致地阐述和更新。强调了血清可交换铜在肝豆状核变性诊断和监测中的地位，并提供了其临床使用的参考范围；新增神经精神症状对症治疗的药物推荐和总结；还增加了儿童患者向成人管理过渡的问题，专门讨论了过渡计划的启动时间、参与团队成员以及具体措施等。这些更新充分体现了肝豆状核变性诊疗领域的最新证据和临床需求的发展。本文对指南中的推荐意见进行摘译。

In April 2025，the European Association for the Study of the Liver （EASL） released an updated edition of clinical practice guidelines on Wilson’s disease，and compared with the 2012 edition，the updated guidelines perform detailed elaboration and updates on clinical manifestations，diagnostic algorithms，treatment strategies，and monitoring protocols，emphasize the role of serum exchangeable copper in the diagnosis and monitoring of Wilson’s disease，and provide the reference ranges for clinical application. The updated guidelines also recommend and summarize the drugs for symptomatic treatment of neuropsychiatric symptoms，add a dedicated section on transitioning pediatric patients to adult care，and discuss the optimal timing，multidisciplinary team composition，and implementation frameworks of the transition plan. These updates fully reflect the latest evidence and the development of clinical needs in the diagnosis and treatment of Wilson’s disease. This article gives an excerpt of the recommendations in the guidelines.