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目的探讨肝豆状核变性患者并发自主神经功能障碍的表现。方法回顾性分析2例肝豆状核变性并发自主神经功能障碍患者的临床资料。结果本组2例肝豆状核变性患者存在ECG乏氏试验、皮肤交感反应、卧立位血压异常,提示为自主神经功能障碍。2例患者均在二巯基丙磺钠治疗后自主神经功能障碍症状改善。结论肝豆状核变性患者并发自主神经功能障碍报道少见,其病因尚不明确;经二巯基丙磺钠驱铜相关治疗后症状改善。
Objective To investigate the manifestations of autonomic dysfunction in patients with hepatolenticular degeneration. Methods The clinical data of 2 patients with hepatolenticular degeneration complicated with autonomic dysfunction were retrospectively analyzed. Results The group of 2 patients with hepatolenticular degeneration there ECG ECG test, skin sympathetic reaction, lying position abnormal blood pressure, suggesting autonomic dysfunction. Both patients had improvements in symptoms of autonomic dysfunction after treatment with sodium dimercaptopropanesulfonate. Conclusions Hepatolenticular degeneration complicated by autonomic dysfunction is rare and its etiology is not clear. Symptoms are improved after copper-related therapy with dimercaptopropane sodium.