Clinical Efficacy and Safety of Chelation Treatment with Typical Penicillamine in Cross Combination with DMPS Repeatedly for Wilson's Disease  

作　　者：徐三清 李旭芳 朱慧云 刘艳 方峰 陈玲 

机构地区：[1]Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology [2]Department of Pediatrics, Guangzhou Women and Children’s Medical Center

出　　处：《Journal of Huazhong University of Science and Technology(Medical Sciences)》2013年第5期743-747,共5页华中科技大学学报（医学英德文版）

摘　　要：The aim of this study was to assess the clinical efficacy and safety of chelation treatment with penicillamine （PCA） in cross combination with sodium 2, 3-dimercapto-l-propane sulfonate （DMPS） repeatedly in patients with Wilson＇s disease （WD）. Thirty-five patients with WD were enrolled. They were administrated intravenous DMPS in cross combination with oral PCA alternately which was practiced repeatedly, all with Zinc in the meantime. During the treatment, clinical observations and 24-h urine copper excretion as well as adverse effects of medicines were recorded and analyzed. Although the incidence of adverse effects was not significantly different after either intravenous DMPS or oral PCA treatment, levels of 24-h urine copper tended to be higher after short-term intravenous DMPS than that of oral PCA. Adverse effects in the course of intravenous DMPS were mainly neutropenia, thrombocy- topenia, allergic reaction and bleeding tendency. As compared with oral PCA alone or intravenous DMPS alone, such repeated cross combination treatment could as much as possible avoid continued drug adverse effects or poor curative effect and had less chance to stop treatment in WD patients. Im- proved or recovered liver fimction in 71% of the patients, alleviated neurologic symptoms in 50% of the patients, and disappeared hematuria in 70% of the patients could be observed during the follow-up pe- riod of 6 months to 5 years after such combined chelation regimen. Chelation treatment repeatedly with oral penicillamine in cross combination with intravenous DMPS alternately could be more beneficial for WD patients to relieve symptoms, avoid continued drug adverse effects and maitain lifelong therapy.The aim of this study was to assess the clinical efficacy and safety of chelation treatment with penicillamine （PCA） in cross combination with sodium 2, 3-dimercapto-l-propane sulfonate （DMPS） repeatedly in patients with Wilson＇s disease （WD）. Thirty-five patients with WD were enrolled. They were administrated intravenous DMPS in cross combination with oral PCA alternately which was practiced repeatedly, all with Zinc in the meantime. During the treatment, clinical observations and 24-h urine copper excretion as well as adverse effects of medicines were recorded and analyzed. Although the incidence of adverse effects was not significantly different after either intravenous DMPS or oral PCA treatment, levels of 24-h urine copper tended to be higher after short-term intravenous DMPS than that of oral PCA. Adverse effects in the course of intravenous DMPS were mainly neutropenia, thrombocy- topenia, allergic reaction and bleeding tendency. As compared with oral PCA alone or intravenous DMPS alone, such repeated cross combination treatment could as much as possible avoid continued drug adverse effects or poor curative effect and had less chance to stop treatment in WD patients. Im- proved or recovered liver fimction in 71% of the patients, alleviated neurologic symptoms in 50% of the patients, and disappeared hematuria in 70% of the patients could be observed during the follow-up pe- riod of 6 months to 5 years after such combined chelation regimen. Chelation treatment repeatedly with oral penicillamine in cross combination with intravenous DMPS alternately could be more beneficial for WD patients to relieve symptoms, avoid continued drug adverse effects and maitain lifelong therapy.

关 键 词：Wilson＇s disease sodium 2  3-dimercapto-l-propane sulfonate PENICILLAMINE efficacy adverse effects 

分 类 号：R741[医药卫生—神经病学与精神病学]