临床肝胆病杂志2026，Vol.42Issue(3)：502-508,7.DOI:10.12449/JCH260302
回顾和展望:全球肝豆状核变性诊治共识/指南的异同
Similarities and differences in the diagnosis and treatment of Wilson disease across global consensus statements/guidelines:Retrospect and prospect
摘要
Abstract
This article systematically reviews and compares the major international English consensus statements/guidelines on the diagnosis and treatment of Wilson disease published since 2022,with a focus on the recommendations from multidisciplinary expert consensus statements/guidelines.These consensus statements/guidelines mainly include the multidisciplinary treatment guidelines issued by the American Association for the Study of Liver Diseases in 2022,the clinical practice guidelines released by the European Union(European Association for the Study of the Liver/European Reference Network)in 2025,and the practice guidelines published by the British Association for Studies of the Liver in 2022,and comparative analysis and summarization were performed with reference to the 2025 edition of Chinese Multidisciplinary Expert Consensus on Orphan/Anticopper Drugs and Other Non-drug Management of Hepatolenticular Degeneration(CMEC-HLD).Overall,the core content remained basically consistent between the guidelines of the European Union,the US,and the UK and CMEC-HLD,while many details varied due to the differences in experiences and research advances across these countries.Globally,there is still a lack of truly meaningful medical guideline for Wilson disease driven by evidence-based medicine,which requires further research and international cooperation among peers in the future.关键词
肝豆状核变性/诊断/治疗学/指导方针(主题)Key words
Hepatolenticular Degeneration/Diagnosis/Therapeutics/Guidelines as Topic引用本文复制引用
王晓平..回顾和展望:全球肝豆状核变性诊治共识/指南的异同[J].临床肝胆病杂志,2026,42(3):502-508,7.基金项目
国家自然科学基金(81671103) （81671103）
国际帕金森病暨运动障碍协会罕见病资助项目(MDS 2017&2022) （MDS 2017&2022）
上海交通大学医学院转化医学创新中心资助项目(2015-1) National Natural Science Foundation of China(81671103) （2015-1）
Rare Program of Developing World Education(DWEP),International Parkinson's Disease&Movement Disorders Society(MDS 2017&2022) （DWEP）
Innovation Center of Translational Medicine,Shanghai JiaoTong University Medical School(2015-1) （2015-1）
