title: Long-term effects of a combination of D-penicillamine and zinc salts in the treatment of Wilson's disease in children.
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.
authors.affiliation: Department of Pediatrics, Affiliated Hospital of Medical College, Qingdao University, Qingdao 266003;
authors.forename: Hong
authors.initials: H
authors.lastname: Chang
authors.forename: Aijing
authors.initials: A
authors.lastname: Xu
authors.forename: Zhihong
authors.initials: Z
authors.lastname: Chen
authors.forename: Ying
authors.initials: Y
authors.lastname: Zhang
authors.forename: Fei
authors.initials: F
authors.lastname: Tian
authors.forename: Tang
authors.initials: T
authors.lastname: Li
keywords: D-penicillamine; Wilson’s disease; child; zinc sulfate