Zhang Jing, Li Liangyong, Chen Huaizhen, Yang Wenming
Department of Neurology, The First Affiliated Hospital of Anhui University of Traditional Chinese Medicine, Hefei 230031, China.
J Tradit Chin Med. 2018 Feb;38(1):89-94.
To investigate the effect and safety of Gandouling plus low-dose D-penicillamine for treating patients with Wilson's disease (WD) who have neurological symptoms.
WD patients with neurological symptoms were divided into two groups: a treatment group (n = 53) and a control group (n = 50). The treatment group received anti-copper therapy with a combination of Gandouling and low-dose D-penicillamine (10 mg/kg), whereas the control group was with conventional dose D-penicillamine (20 mg/kg) monotherapy. The clinical efficacies, adverse reactions, and results of the various hematological and biochemical investigations were recorded and analyzed statistically.
Overall, 98.11% of the WD patients treated with the combined therapy experienced alleviation of their neurological condition (paralleled by a significantly improved Global Assessment Scale score or remained stable). Their white blood cell and platelet counts stabilized, and their liver function was improved or remained stable. The combined therapy also obviously promoted improved 24-h urinary copper excretion. Only 15.09% of the WD patients with the combined therapy experienced adverse reactions, including neurological deterioration in one case (1.89%) and hepatic worsening in one case (1.89%), which was less frequent than that in the control group given conventional-dose D-penicillamine monotherapy.
Treating WD patients with neurological symptoms using Gandouling plus low-dose D-penicillamine is effective and safe.
探讨肝豆灵联合小剂量青霉胺治疗有神经症状的肝豆状核变性（WD）患者的疗效及安全性。
将有神经症状的WD患者分为两组：治疗组（n = 53）和对照组（n = 50）。治疗组采用肝豆灵联合小剂量青霉胺（10 mg/kg）进行驱铜治疗，而对照组采用常规剂量青霉胺（20 mg/kg）单药治疗。记录并统计分析临床疗效、不良反应以及各项血液学和生化检查结果。
总体而言，接受联合治疗的WD患者中98.11%的神经状况得到缓解（同时全球评估量表评分显著改善或保持稳定）。他们的白细胞和血小板计数稳定，肝功能得到改善或保持稳定。联合治疗还明显促进了24小时尿铜排泄的改善。接受联合治疗的WD患者中只有15.09%出现不良反应，包括1例神经功能恶化（1.89%）和1例肝功能恶化（1.89%），其发生率低于接受常规剂量青霉胺单药治疗的对照组。
肝豆灵联合小剂量青霉胺治疗有神经症状的WD患者有效且安全。
