title: Clinical efficacy and safety of chelation treatment with typical penicillamine in cross combination with DMPS repeatedly for Wilson's disease.
abstract: 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-1-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, thrombocytopenia, 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. Improved or recovered liver function 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 period 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 maintain lifelong therapy.
authors.affiliation: Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
authors.forename: San-Qing
authors.initials: SQ
authors.lastname: Xu
authors.affiliation: Department of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou, 510120, China.
authors.forename: Xu-Fang
authors.initials: XF
authors.lastname: Li
authors.affiliation: Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
authors.forename: Hui-Yun
authors.initials: HY
authors.lastname: Zhu
authors.affiliation: Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
authors.forename: Yan
authors.initials: Y
authors.lastname: Liu
authors.affiliation: Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
authors.forename: Feng
authors.initials: F
authors.lastname: Fang
authors.affiliation: Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China. chenling@tjh.tjmu.edu.cn.
authors.forename: Ling
authors.initials: L
authors.lastname: Chen
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D002614:Chelating Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D015913:Chelation Therapy / Q000009:adverse effects / Q000379:methods*; D002648:Child; D003300:Copper / Q000652:urine; D004334:Drug Administration Schedule; D004342:Drug Hypersensitivity / Q000209:etiology; D004359:Drug Therapy, Combination; D006527:Hepatolenticular Degeneration / Q000188:drug therapy*; D006801:Humans; D007275:Injections, Intravenous; D008297:Male; D009503:Neutropenia / Q000139:chemically induced; D010314:Partial Thromboplastin Time; D010396:Penicillamine / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D011517:Prothrombin Time; D013921:Thrombocytopenia / Q000139:chemically induced; D013997:Time Factors; D016896:Treatment Outcome; D014494:Unithiol / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*
chemical_list: D002614:Chelating Agents; D014494:Unithiol; D003300:Copper; D010396:Penicillamine