Xu Meng-Bei, Rong Pei-Qing, Jin Ting-Yu, Zhang Pei-Pei, Liang Hai-Yong, Zheng Guo-Qing
Department of Neurology, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, China.
Front Pharmacol. 2019 Mar 29;10:277. doi: 10.3389/fphar.2019.00277. eCollection 2019.
Wilson's disease (WD) is a rare autosomal recessive inherited disorder of chronic copper toxicosis. Currently, Chinese herbal medicines (CHM) is widely used for WD. Here, we conducted an updated systematic review to investigate the efficacy and safety of CHM for WD and its possible mechanisms. Randomized-controlled clinical trials (RCTs), which compared CHM with Western conventional medicine or placebo for WD, were searched in six databases from inception to July 2017. The methodological quality was assessed using 7-item criteria from the Cochrane's collaboration tool. All the data were analyzed using Rev-Man 5.3 software. Eighteen studies involving 1,220 patients were identified for the final analyses. A score of study quality ranged from 2/7 to 4/7 points. Meta-analyses showed that CHM could significantly increase 24-h urinary copper excretion and improve liver function and the total clinical efficacy rate for WD compared with control ( < 0.05). Additionally, CHM was well tolerated in patients with WD. The underlying mechanisms of CHM for WD are associated with reversing the ATP7B mutants, exerting anti-oxidation, anti-inflammation, and anti-hepatic fibrosis effects. In conclusion, despite the apparent positive results, the present evidence supports, to a limited extent because of the methodological flaws and CHM heterogeneity, that CHM paratherapy can be used for patients with WD but could not be recommended as monotherapy in WD. Further rigorous RCTs focusing on individual CHM formula for WD are warranted.
威尔逊病(WD)是一种罕见的常染色体隐性遗传性慢性铜中毒疾病。目前,中药被广泛用于治疗WD。在此,我们进行了一项更新的系统评价,以研究中药治疗WD的疗效、安全性及其可能的作用机制。检索了6个数据库,从建库至2017年7月,纳入比较中药与西药或安慰剂治疗WD的随机对照临床试验(RCT)。采用Cochrane协作工具的7项标准评估方法学质量。所有数据均使用Rev-Man 5.3软件进行分析。最终分析纳入18项研究,共1220例患者。研究质量评分范围为2/7至4/7分。Meta分析表明,与对照组相比,中药能显著增加24小时尿铜排泄量,改善肝功能及WD的总临床有效率(P<0.05)。此外,WD患者对中药耐受性良好。中药治疗WD的潜在机制与逆转ATP7B突变体、发挥抗氧化、抗炎和抗肝纤维化作用有关。总之,尽管有明显的阳性结果,但由于方法学缺陷和中药的异质性,目前的证据在有限程度上支持中药辅助治疗可用于WD患者,但不推荐作为WD的单一疗法。有必要开展针对WD单一中药方剂的进一步严格RCT。