肝豆状核变性患者合并出汗障碍的临床研究

Clinical Research of Dyshidrosis in Patients with Hepatolenticular Degeneration

目的:研究肝豆状核变性(HLD)患者出汗障碍的特点、皮肤交感反应的变化。方法:对66例确诊为HLD患者出汗情况进行调查并行皮肤交感反应的检查。结果:66例HLD患者有36例有出汗障碍;主要表现为四肢远端出汗减少(26例)合并代偿性局部出汗增多。皮肤交感反应异常表现为潜伏期延长、波幅降低甚至不能引出。结论:HLD患者存在明显的出汗障碍以及皮肤交感反应异常。

Objective To comprehend the characteristic of dyshidrosis and change of sympathetic skin responses in hepatolenticular degeneration （HLD） patients.Methods 66 patients with, HLD was investigated in information ofsweeting and examined with SSR.Results Dyshidrosis was found in 36 patients among the 66 patients with HLD,which most manifested decreased sweating in distal end of extremities combining compensating partly increased sweeting, Abnormal appearances of SSR were prolong of latency period and lower of wave amplitude,and even could not be elicited.Conclusion Visible dyshidrosis and abnormal SSR exests in patients with HLD.