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22例确诊肝豆状核变性患者口服二巯丁二酸(DMSA)胶囊自身对照驱铜治疗4wk,剂量:成人4g/d,儿童2g/d,得出疗后平均24h尿铜(18±10μmol)较疗前(4.5±1.9μmol)显著增高(P<0.01),约4倍,而血清和脑脊液铜含量无明显变化。对此体液锌含量无影响。血清巯基浓度疗前(1.07±0.24μmol/L)和疗后各周(分别为1.21±0.26,1.3±0.3,1.19±0.23,1.38±0.21μmol/L)比较差别显著(P<0.05,P<0.01,P<0.05,P<0.01),脑脊液中浓度疗后(0.27±0.11μmol/L)远高于疗前(0.18±0.12μmol/L)(P<0.01)。血清铜蓝蛋白值治疗前后无显著差别。临床观察口服DMSA作用优于静注Na-DMS。
Twenty-two patients with hepatolenticular degeneration were treated with floxane (DMSA) for 4wk for 4wk, the dosage was 4g / d in adults and 2g / d in children, and the average 24h urinary copper (18 ± 10μmol ) Was significantly higher than that before treatment (4.5 ± 1.9μmol) (P <0.01), about 4 times, but the content of copper in serum and cerebrospinal fluid had no significant change. This body fluid zinc content had no effect. Serum sulfhydryl concentration before treatment was significantly higher than that before treatment (1.07 ± 0.24μmol / L) and after treatment (1.21 ± 0.26,1.3 ± 0.3,1.19 ± 0.23,1.38 ± 0.21μmol / L, P < 0.01, P <0.05, P <0.01). The concentration of CSF in cerebrospinal fluid (0.27 ± 0.11μmol / L) was significantly higher than that before treatment (0.18 ± 0.12μmol / L) Serum ceruloplasmin before and after treatment no significant difference. Clinical observation of oral DMSA is better than intravenous Na-DMS.