肝豆状核变性患者肌肉铜、锌、铁、镁含量及二巯基丁二酸钠疗效探讨

Muscle copper,zinc,iron and magnesium levels in patients with hepatolenticular de-generation and effect of DMS.

本文对２５例肝豆状核变性（ＨＬＤ）患者的腓肠肌铜、锌、铁、镁含量及二琉基丁二酸钠（ＤＭＳ）疗效进行了研究。２５例ＨＬＤ脓肠肌铜含量显著高于对照组（Ｐ＜０．００１），锌、铁、镁无明显差异（Ｐ＞０．０５）。ＤＭＳ治疗在１１例患者中虽引起部分症状加重或一过性加重，但按症状出现及好转例次统计，好转率为８３．６２％。治疗后自第二疗程始２４小时尿铜显著增高（Ｐ＜０．０５）；而阶肠肌铜含量轻度增高（Ｐ＞０．０５），且与症状加重高度相关，因此认为ＤＭＳ在增加尿排铜的同时可能引起体内铜离子的再分布。

We studied the contents of gastrocnemius copper,zinc,iron and magnesium and the effect of DMS in 25 patients with hepatolenticular degeneration(HLD).The content of HLD gastrocnemius copper was significantly higher than that in the controls(P<0.001),but no obvious diifference among zinc,iron and magnesium(P>0.05).Effect of DMS was studied in patients,although some symptoms deteriorated,or became temporarily worse with the initiation of therapy, the rusult showed 8.2%improved according to the appear-ing of symptoms and improvement order。From the beginning of the second course of treat-ment 24 hours urinary copper significantly increased(F=2.3714,P<0.05),and the mean garstrocnemius copper content was slighily higher than that before treatment.For increase of gastrociiemius copper highly correlated with deterioration of symptoms,so it is suggeisted that DMS niight lead to new distribution of copperion in the body。