二巯基丙磺酸钠驱铜治疗对肝豆状核变性铜代谢的影响

Study on The Influence on Copper Metabolism in Patients with Hepatolenticular Degeneration Treated by Sodium Dimercaptosulphonate

目的 :探讨驱铜治疗对肝豆状核变性 (HLD)患者铜代谢的影响。方法 :对 12 5例HLD进行了二巯基丙磺酸钠 (DMPS)治疗前后的铜代谢指标检测对比 ,旨在了解驱铜治疗对血清铜蓝蛋白 (CP)、血清铜氧化酶 (SCO)、血清铜 (SCu)及 2 4小时尿铜 (UCu)的影响。结果 :治疗前患者CP、SCO及SCu均较对照组显著降低 (P<0 .0 1) ,三者呈正相关。治疗后患者SCO、SCu较疗前显著降低 (P <0 .0 5～ 0 .0 1) ,治疗时UCu较疗前显著增高(P <0 .0 1) ,8疗程后间歇期UCu较疗前显著减低 (P <0 .0 1)。结论 :HLD患者CP、SCO及SCu明显低于正常人 ,治疗后CP变化不大 ,SCO、SCu降低 。

Objective To investigate the influence on copper metabolism in patients with hepatolenticular degeneration(HLD) treated by sodium dimercaptosulphonate(DMPS). Methods The changes of parameters of copper metabolism including serum ceruloplasmin(CP)、 serum copper oxydase(SCO)、 serum copper(SCu) and urinary copper in 24 hours(UCu) in 128 HLD patients were observed before and after treatment with DMPS. Results The levels of serum Cp、 SCO and SCu HLD patients wre significantly lower than those in the controls before therapy (P<0.01), the content of Ucu was greatly increased during therapy (P<0.01).After therapy, the serum levels of SCO、 Scu were insignificantly further decreased (P<0.05～0.01) and the content of Ucu was greatly lowered after finishing the 8 courses of the treatment (P<0.01). Conclusion The serum levels of Cp、 SCO and SCu in HLD patients were significantly lower than those in the controls, the level of SCO、 SCu would be lowered after therapy. DMPS could significantly promote excretion of urinary copper in the patients.