[The role of zinc in the initial treatment of Wilson's disease in children].
Wilson's disease (WD) is an inherited disease of copper accumulation, caused by a failure of biliary excretion of excess copper. Accumulated copper causes tissue damage. The chelating drugs penicillamine and trientine have been the mainstay of therapy and most patients with WD were treated with the potentially toxic cupriuretic agents. A more recent approach has used zinc, which blocks the absorption of copper and increases copper excretion in the stool, and long term administration induces a negative copper balance. Until recently, most patients have been treated initially with cupriuretic agents to remove excess of copper, and then maintained with oral zinc. Recently, zinc has been used for initial treatment as well and for treatment of the presymptomatic patients. So far, zinc therapy has demonstrated exceptional efficacy and lack of toxicity. In this article we present our data on the long-term follow-up of three children with WD, whose initial as well as consecutive treatment was zinc sulphate. The results demonstrate the efficacy of zinc therapy in treating the presymptomatic patient and in initial treatment of symptomatic children with WD. Our data also indicate low toxicity. However, pediatric patients must be closely monitored due to tendency to stop the treatment when becoming asymptomatic.
Referentni centar za djecju gastroenterologiju i prehranu, Klinika za djecje bolesti Zagreb.
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M
Cacić
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Percl
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O
Jadresin
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Kolacek
D000293:Adolescent; D002648:Child; D003300:Copper / Q000378:metabolism; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000378:metabolism; D006801:Humans; D008297:Male; D015032:Zinc / Q000627:therapeutic use*
D003300:Copper; D015032:Zinc