摘要
28例肝豆状核变性患者采用二巯基丁二酸钠(Na-DMS)静脉冲击疗法,成人剂量1.0~2.0g/次,儿童0.5~1.0g/次,共8周。结果:26例临床症状改善,2例无效,以假性硬化型和肝豆状核变性型疗效好。疗后平均24小时尿铜比疗前明显增高(P<0.01),疗后各周的尿排铜量与时间呈负相关(r=-0.806,P<0.02)。该药对血、尿、脑脊液的锌、铁、钙含量无明显影响。11例出现齿龈及鼻粘膜渗血,少数病例出现恶心及发热、皮疹等过敏反应。
NaDMS were given to 28 patients with hepatolenticular degeneration at the dosage of 1.0~2.0g/ time for adults and 0.5~1.0g/time for children by intravenously injection for 8 weeks.The results showed that 26 cases were improved, 2 cases were not effective.The effect on the type of pseudosclerosis and Wilson's was better than that of other medication. The excration of urine copper was significant higher than that of premedication (P<0.01), and was negative correlation with the time (r=-0.806, P<0.02), The drug had no influence on the content of zinc, ferrum, calcium in blood, urine, and CSF.During the treatment,11 patients had gingival bleeding and rhinorrhagia,a few patients had nausea, decreased food intake, and allergic reaction.This report demonstrated that NaDMS is a more powerful and less toxicity a bypotoxicity copperchelating agent.
出处
《临床神经病学杂志》
CAS
1998年第3期152-155,共4页
Journal of Clinical Neurology
关键词
肝豆状核变性
微量元素
二巯基丁二酸钠
铜
Hepatolenticular degeneration Trace element Soudium dimercaptosucinate Copper