二巯丁二酸胶囊对肝豆状核变性的治疗评价

Therapeutic assessment of dimercaptosuccinic acid capsule in the treatment of hepatolenticular degeneration

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。

DMSA capsules were given to 22 patients of hepato-lenticular degeneration at the dosage of 4 g/d for adults and 2 g/d for children orally for 4 wk. The results indicated that the average 24 h urinary excretion of copper after medication (18+ 10 mol) were significantly higher than that of premedication (4.5 h 1.9 umol; P<0.01), but no changes of copper contents occurred in serum and cerebrospinal fluid(CSF). The zinc content in the body fluid did not change after medication.The thiol concentration in the serum were higher weekly after medication(1.21+0.26,1.3+01.3,1.19+0.23,1.38+0.21umol/L)than premedication (1.07+0.24umol/L) with significant differences (P<0.05, 0.01, 0.05, 0.01 respectively), and this concentration in CSF after medication for 4 wk (0.27+0.11 umol/L) were also higher than that of premedication (0.18+0. 12umol/L; P< OiOl), 'DMSA did not raise ceruloplasmin level. Clinical observation revealed that DMSA is better than Na-DMS for the treatment of this disease.