口服二巯丁二酸对肝豆状核变性临床疗效及尿铜的影响

Copper excretion in 20 cases with hepatolenticular degeneration treated by DMSA

２０例肝豆状核变性（ＨＬＤ）患者，男１１例，女９例。年龄１０～３７岁（平均２０±６．４岁）。口服二巯丁二酸（ＤＭＳＡ）７０ｍｇ／ｋｇ．ｄ，观察８周，结果表明：７例显效，１２例好转，１例无效，有效率达９５％。疗前与疗后１～８周２４ｈ尿铜比较，结果显示：疗后各周及疗后总平均尿铜值均显著高于疗前。其尿排铜增高与临床疗效相一致。治疗期６例出现齿龈出血、皮下瘀斑外，未见其他副反应。说明ＤＭＳＡ是治疗ＨＬＤ的一种较理想的驱铜药物。

Abstract Copper excretion in 2 0 cases with hepatolenticular degeneration(HLD)treated by DMSA isreported.It included 11 males and 9 females with age ranging from 10 to 37 years(mean age 20±6.4).DMSA capsules were qiven orally at dosage of 70 mg/kg.d for 8 weeds.Results showedthat seven cases(35%)were gignificantly improved,12(60%)improved and one paticnt(5%)was not effective. Increase in urinary copper excretion coincided with clinical improvement.Gingi-val hemorrhage and ecchymosis occurred in 6 patients during treatment.It suggested that DMSA isan effective agent for HLD.