Journal of Huazhong University of Science and Technology [Medical Sciences]

, Volume 33, Issue 5, pp 743–747

Clinical efficacy and safety of chelation treatment with typical penicillamine in cross combination with DMPS repeatedly for Wilson’s disease

Authors

  • San-qing Xu徐三清
    • Department of Pediatrics, Tongji Hospital, Tongji Medical CollegeHuazhong University of Science and Technology
  • Xu-fang Li李旭芳
    • Department of PediatricsGuangzhou Women and Children’s Medical Center
  • Hui-yun Zhu朱慧云
    • Department of Pediatrics, Tongji Hospital, Tongji Medical CollegeHuazhong University of Science and Technology
  • Yan Liu刘 艳
    • Department of Pediatrics, Tongji Hospital, Tongji Medical CollegeHuazhong University of Science and Technology
  • Feng Fang方 峰
    • Department of Pediatrics, Tongji Hospital, Tongji Medical CollegeHuazhong University of Science and Technology
    • Department of Pediatrics, Tongji Hospital, Tongji Medical CollegeHuazhong University of Science and Technology
Article

DOI: 10.1007/s11596-013-1190-z

Cite this article as:
Xu, S., Li, X., Zhu, H. et al. J. Huazhong Univ. Sci. Technol. [Med. Sci.] (2013) 33: 743. doi:10.1007/s11596-013-1190-z

Summary

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 maitain lifelong therapy.

Key words

Wilson’s diseasesodium 2, 3-dimercapto-1-propane sulfonatepenicillamineefficacyadverse effects

Copyright information

© Huazhong University of Science and Technology and Springer-Verlag Berlin Heidelberg 2013