Abstract
A case of a 11-yr-long Wilson’s disease treatment in a 16-yr-old boy with neurologic presentation was analyzed and monitored. In the face of severe symptoms of chelator intolerance, a comparatively low dose of 100 mg of zinc has been administered for the entire 11-yr-long treatment. Considerable improvement of clinical status was achieved, with accompanying regression of central nervous system lesion. The parameters of copper metabolism were normalized with effective urine elimination. The low-dose oral zinc intake proved to be therapeutically effective, eliminating further copper tissue toxicity.
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References
G. J. Brewer and V. Yuzbasiyan-Gurkan, Wilson disease, Medicine 71, 136–164 (1992).
P. C. Bull, G. R. Thomas, J. M. Rommens, et al., The Wilson disease gene is a putative copper transporting P-type ATP-ase similar to the Menkes gene, Nat. Genet. 5, 327–329 (1993).
I Chernov, M. Devoto, L. M. Brzustowicz, et al., Mapping, cloning and genetic characterization of the region containing the Wilson disease gene, Nat. Genet. 5, 338–339 (1993).
W. Stremmel, Pathogenese des Morbus Wilson, Z. Gastroenterol. 30, 199–201 (1992).
K. W. Meyerrose, C. Niederau, W. Stremmel, et al., Wilson’s disease: clinical presentation, treatment and survival, Ann. Intern. Med. 115, 720–723 (1991).
I. Sternlieb, The outlook for the diagnosis of Wilson’s disease, J. Hepatol. 17, 263–265 (1993).
G. J. Brewer, Practical recommendations and new therapies for Wilson’s disease, Drugs 50, 240–242 (1995).
C. Smolarek and W. Stremmel, Therapie des Morbus Wilson, Z. Gastroenterol. 37, 293–300 (1999).
E. W. Rice, Standarisation of ceruloplasmin activity in terms of international units. Oxidative formation of Bandrowski base from p-phenylenediamine by ceruloplasmin, Ann. Biochem. 3, 452–456 (1962).
V. Yuzbasiyan-Gurkan, A. Brider, T. Nostrant, R. J. Cousins, and G. J. Brewer, The treatment of Wilson’s disease with zinc. Intestinal metallothionein induction, J. Lab. Clin. Med. 120, 380–386 (1992).
G. C. Sturniolo, C. Mestriner, P. Irato, B. S. Albergoni, G. Longo, and R. D’Inca, Zinc treatment increases duodenal concentrations of metallothionein and iron in Wilson’s diasease patients, 94, 334–338 (1999).
P. W. F. Fischer, A. Giroux, and M. R. L’Abre, The effect of dietary zinc on intestinal copper absorption, Am. J. Clin. Nutr. 34, 1670–1675 (1981).
L. Rossaro, G. C. Sturniolo, G. Giacon, et al., Zinc therapy in Wilson’s disease—observations in five patients, Am. J. Gastroenterol. 85, 665–668 (1990).
G. J. Brewer, V. Yuzbasiyan-Gurkan, and R. Dick, Zinc therapy of Wilson’s disease; VIII—dose response studies, J. Trace Elements Exp. Med. 3, 227–231 (1990).
G. J. Brewer, G. M. Hill, A. S. Prasad, et al. The treatment of Wilson’s disease with zinc; IV. Efficacy monitoring using urine and plasma copper, Proc. Soc. Exp. Biol. Med. 7, 446–449 (1987).
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Najda, J., Stella-Hołowiecka, B. & Machalski, M. Low-dose zinc administration as an effective Wilson’s disease treatment. Biol Trace Elem Res 80, 281–284 (2001). https://doi.org/10.1385/BTER:80:3:281
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DOI: https://doi.org/10.1385/BTER:80:3:281