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- W2073302179 abstract "Objectives: Wilson's disease (WD) is an inherited disorder of copper metabolism characterised by a failure of biliary excretion of copper. It is generally accepted that “presymptomatic” patients diagnosed in childhood should be treated prophylactically with zinc. Methods: Here we report our results in 22 children with continuous oral zinc therapy over ten years. Zinc sulfate was used at a dose of 25 mg b.i.d. until the age of 6 years, 25 mg t.i.d. from 7 to 16 years or to a body weight of 125 lbs, 50 mg t.i.d.thereafter. Results: Five years after starting zinc treatment an highly significant lowering of alanine aminotransferase (ALT), aspartate aminotransferase (AST) and urinary copper excretion was observed, whereas white blood cells did not vary significantly. 6 out of 22 patients continued to have ALT above normal and only one patient showed ALT more than 1.5 times the upper normal limit. Further lowering of ALT, AST and urinary copper excretion was observed at the end of the 10-year follow-up. All histological scores were significantly decreased after treatment. Conclusion: The excellent clinical results in all our patients, coupled with the improvement in liver histology in the vast majority, indicate convincingly that zinc treatment can control the disease effectively and safely, preventing its progression over 10 years. Therefore, our findings indicate that pediatric presymptomatic patients should be treated with zinc as the treatment of choice." @default.
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- W2073302179 date "2005-05-01" @default.
- W2073302179 modified "2023-10-14" @default.
- W2073302179 title "ZINC AS SOLE FIRST LINE THERAPY IN CHILDREN WITH WILSON??S DISEASE: TEN YEARS FOLLOW UP STUDY" @default.
- W2073302179 doi "https://doi.org/10.1097/00005176-200505000-00209" @default.
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