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

  • San-qing Xu (徐三清)
  • Xu-fang Li (李旭芳)
  • Hui-yun Zhu (朱慧云)
  • Yan Liu (刘 艳)
  • Feng Fang (方 峰)
  • Ling Chen (陈 玲)
Article

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 disease sodium 2, 3-dimercapto-1-propane sulfonate penicillamine efficacy adverse effects 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Ala A, Walker AP, Ashkan K, et al. Wilson’s disease. Lancet, 2007,369(9559):397–408PubMedCrossRefGoogle Scholar
  2. 2.
    Brewer GJ. Novel therapeutic approaches to the treatment of Wilson’s disease. Expert Opin Pharmacother, 2006,7(3):317–324PubMedCrossRefGoogle Scholar
  3. 3.
    Brewer GJ, Terry CA, Aisen AM, et al. Worsening of neurologic syndrome in patients with Wilson’s disease with initial penicillamine therapy. Arch Neurol, 1987,44(5):490–493PubMedCrossRefGoogle Scholar
  4. 4.
    Subramanian I, Vanek ZF, Bronstein JM. Diagnosis and treatment of Wilson’s disease. Curr Neurol Neurosci Rep, 2002,2(4):317–323PubMedCrossRefGoogle Scholar
  5. 5.
    Linn F, Houwen R, van Hattum J, et al. Long-term exclusive zinc monotherapy in symptomatic Wilson disease: experience in 17 patients. Hepatology, 2009,50(5):1442–1452PubMedCrossRefGoogle Scholar
  6. 6.
    Purchase R. The treatment of Wilson’s disease, a rare genetic disorder of copper metabolism. Sci Prog, 2013,96 (Pt 1):19–32CrossRefGoogle Scholar
  7. 7.
    Sinha S, Taly AB. Withdrawal of penicillamine from zinc sulphate-penicillamine maintenance therapy in Wilson’s disease: promising, safe and cheap. J Neurol Sci, 2008,264(1-2): 129–132PubMedCrossRefGoogle Scholar
  8. 8.
    Ping CC, Hassan Y, Aziz NA, et al. Discontinuation of penicillamine in the absence of alternative orphan medicines (trientine-zinc): a case of decompensated liver cirrhosis in Wilson’s disease. J Clin Pharm Ther, 2007, 32(1):101–107PubMedCrossRefGoogle Scholar
  9. 9.
    Wang XP, Yang RM, Ren MS, et al. Anticopper efficacy of captopril and sodium dimercaptosulphonate in patients with Wilson’s disease. Funct Neurol, 2003,18(3):149–153PubMedGoogle Scholar

Copyright information

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

Authors and Affiliations

  • San-qing Xu (徐三清)
    • 1
  • Xu-fang Li (李旭芳)
    • 2
  • Hui-yun Zhu (朱慧云)
    • 1
  • Yan Liu (刘 艳)
    • 1
  • Feng Fang (方 峰)
    • 1
  • Ling Chen (陈 玲)
    • 1
  1. 1.Department of Pediatrics, Tongji Hospital, Tongji Medical CollegeHuazhong University of Science and TechnologyWuhanChina
  2. 2.Department of PediatricsGuangzhou Women and Children’s Medical CenterGuangzhouChina

Personalised recommendations