title: Metal element excretion in 24-h urine in patients with Wilson disease under treatment of D-penicillamine.
abstract: Wilson disease is an inherited autosomal recessive disorder causing copper accumulation and consequent toxicity. D-Penicillamine, a potent metal chelator, is an important therapy for Wilson disease. To investigate the changes of metal elements under the treatment of D-penicillamine, we determined the levels of Cu, Zn, Mg, Ca, Fe, Se, Mn, Pb, Hg, Cd, As, Tl, and Al by ICP-MS in 24-h urine of 115 Wilson disease patients who had received treatment with D: -penicillamine for 1 month to 22 years at maintenance doses, as well as 115 age-matched, healthy controls. The levels of Cu, Mg, Ca, Zn, Hg, Pb, Tl, Cd, and Mn in the 24-h urine of the cases were significantly higher than those of the controls (P < 0.05), and the observed increases in the levels of Mg, Ca, and Zn were directly correlated with the treatment duration with Pearson Correlation Coefficient (R) of 0.356 (Mg), 0.329 (Ca), and 0.313 (Zn), respectively (P < 0.05). On the other hand, the levels of Al and As in the 24-h urine were lower than those of the controls (P < 0.05) and were negatively correlated with the treatment time with R of -0.337 (Al) and -0.398 (As), respectively, (P < 0.05). Thus, this study indicates that the levels of metal elements may be altered in patients with Wilson disease under the treatment of D-penicillamine.
authors.affiliation: Department of Pediatrics, XinHua Hospital affiliated to Shanghai Jiao Tong University School of Medicine, 1665 Kongjiang Road, Shanghai, China.
authors.forename: Lisu
authors.initials: L
authors.lastname: Huang
authors.forename: Xiaodan
authors.initials: X
authors.lastname: Yu
authors.forename: Jun
authors.initials: J
authors.lastname: Zhang
authors.forename: Xiaoqing
authors.initials: X
authors.lastname: Liu
authors.forename: Yongjun
authors.initials: Y
authors.lastname: Zhang
authors.forename: Xianting
authors.initials: X
authors.lastname: Jiao
authors.forename: Xiaogang
authors.initials: X
authors.lastname: Yu
mesh_terms: D000293:Adolescent; D000328:Adult; D002614:Chelating Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000652:urine*; D006801:Humans; D008297:Male; D008670:Metals / Q000652:urine*; D008875:Middle Aged; D010396:Penicillamine / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D002614:Chelating Agents; D008670:Metals; D010396:Penicillamine