关键词: 肝豆状核变性, 平衡障碍, 湿热, 治疗结果

Abstract: Background　Wilson disease（WD）is an autosomal recessive inherited disorder of copper metabolism，and its pathologlical basis is the deposition of a large amount of copper due to copper metabolic disorder caused by ATP7B gene mutation，and common features of this disease include progressive liver disease and neurological symptoms.Western medicine treatments have no significant therapeutic effects on such patients，so many scholars explore the treatment with integrated traditional Chinese and Western medicine in order to improve the efficacy．Objective　To study the efficacy of Gandou Decoction combined with copper removal therapy in the treatment of balance disorder in patients with interior retention of damp-heat WD.Methods　We enrolled 60 cases of WD with interior intention of damp-heat syndrome and balance disorder who received inpatient treatment in Encephalopathy Center，the First Affiliated Hospital of Anhui University of Chinese Medicine from 2017 to 2018，and divided them into the observation group and the control group with 30 in each based on a random number table.Removing copper with intravenous drip of Sodium Dimercaptopropionate Injection was given to both groups，and oral administration of Gandou Decoction was additionally given to the observation group，for 32 days〔4 courses of treatment（8-day treatment was defined as a course）〕．Pre- and post-treatment balance function were assessed by the Berg Balance Scale（BBS），pre- and post-treatment TCM syndrome score and 24-hour urinary copper excretion were collected.Results　After treatment，the mean score of BBS was significantly higher than that before treatment in both groups（P<0.05），especially in the observation group（P<0.05）.The mean TCM syndrome score was significantly lower than that before treatment in both groups（P<0.05），especially in the observation group（P<0.05）.The mean 24-hour urinary copper excretion in both groups was significantly higher than that before treatment（P<0.05），especially in the observation group（P<0.05）.Conclusion　Gandou Decoction combined with copper removal therapy may significantly improve the balance disorder，relieve the clinical symptoms and improve the quality of life in patients with interior retention of damp-heat WD.

Key words: Hepatolenticular degeneration, Balance disorder, Dampness-heat, Treatment outcome