Long-term effects of a combination of D-penicillamine and zinc salts in the treatment of Wilson's disease in children

Chang,Hong; Xu,Aijing; Chen,Zhihong; Zhang,Ying; Tian,Fei; Li,Tang

doi:10.3892/etm.2013.971

April 2013 Volume 5 Issue 4

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April 2013 Volume 5 Issue 4

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Article

Long-term effects of a combination of D-penicillamine and zinc salts in the treatment of Wilson's disease in children

Authors:
- Hong Chang
- Aijing Xu
- Zhihong Chen
- Ying Zhang
- Fei Tian
- Tang Li
View Affiliations / Copyright

Affiliations: Department of Pediatrics, Affiliated Hospital of Medical College, Qingdao University, Qingdao 266003, P.R. China, Children's Hospital, Guangzhou Women and Children's Medical Center, Guangzhou Medical College, Guangzhou 510520, P.R. China
Pages: 1129-1132
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Published online on: February 22, 2013

https://doi.org/10.3892/etm.2013.971
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Abstract

The aim of this study was to investigate the effectiveness of a high-dose zinc sulfate and low-dose D-penicillamine combination in the treatment of pediatric Wilson's disease (WD). A retropective chart review of 65 patients with WD was conducted. These patients received D-penicillamine (8-10 mg/kg/day) and zinc sulfate as the primary treatment. The pediatric dose of elemental zinc is 68-85 mg/day until 6 years of age, 85-136 mg/day until 8 years of age, 136-170 mg/day until 10 years of age and then 170 mg/day, in 3 divided doses 1 h before meals. After clinical and biochemical improvement or stabilization, zinc sulfate alone was administered as the maintenance therapy. Under treatment, the majority of patients (89.2%) had a favourable outcome and 3 patients succumbed due to poor therapy compliance. No penicillamine-induced neurological deterioration was noted and side-effects were observed in <11% of patients over the entire follow-up period. Benefical results on the liver and neurological symptoms were reported following extremely long-term treatment with a combination of low-dose D-penicillamine and high-dose zinc sulfate. Therefore, this regimen is an effective and safe treatment for children with WD.

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Journals

International Journal of Molecular Medicine

International Journal of Oncology

Molecular Medicine Reports

Oncology Reports

Experimental and Therapeutic Medicine

Oncology Letters

Biomedical Reports

Molecular and Clinical Oncology

World Academy of Sciences Journal

International Journal of Functional Nutrition

International Journal of Epigenetics

Medicine International

Long-term effects of a combination of D-penicillamine and zinc salts in the treatment of Wilson's disease in children

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