J Hepatol. 2012 Mar;56(3):671-85. doi: 10.1016/j.jhep.2011.11.007.
This Clinical Practice Guideline (CPG) has been developed to assist physicians and other healthcare providers in the diagnosis and management of patients with Wilson's disease. The goal is to describe a number of generally accepted approaches for diagnosis, prevention, and treatment of Wilson's disease. Recommendations are based on a systematic literature review in the Medline (PubMed version), Embase (Dialog version), and the Cochrane Library databases using entries from 1966 to 2011. The Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) system used in other EASL CPGs was used and set against the somewhat different grading system used in the AASLD guidelines (Table 1A and B). Unfortunately, there is not a single randomized controlled trial conducted in Wilson's disease which has an optimal design. Thus, it is impossible to assign a high or even a moderate quality of evidence to any of the questions dealt with in these guidelines. The evaluation is mostly based on large case series which have been reported within the last decades.
本临床实践指南（CPG）旨在帮助医生和其他医疗保健提供者诊断和治疗威尔逊病患者。目的是描述一些被广泛接受的诊断、预防和治疗威尔逊病的方法。建议是基于对 Medline（PubMed 版本）、Embase（Dialog 版本）和 Cochrane 图书馆数据库中 1966 年至 2011 年的文献进行系统评价得出的。本指南使用了 EASL CPG 中使用的推荐分级的评估、制定与评价（GRADE）系统，并针对 AASLD 指南中使用的略有不同的分级系统（表 1A 和 B）进行了调整。不幸的是，在威尔逊病中没有进行过一项设计最佳的随机对照试验。因此，对于这些指南中涉及的任何问题，都不可能给予高甚至中等质量的证据。评估主要基于过去几十年报告的大型病例系列。
