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| ZINC SUPPLEMENTATION IN WILSON DISEASE |
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As per Wilson’s Disease Association International:
Zn acetate (Zinconia) is FDA approved drug in treatment of Wilson Disease. Zinc acts by blocking the absorption of copper in the intestinal tract. This action both depletes accumulated copper and prevents its re-accumulation. Zinc's effectiveness has been shown by more than 30 years of considerable experience overseas. A major advantage of zinc therapy is its lack of side effects.
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REFERENCE 1
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Abstract:
Wilson Disease in Children:
Journal of Pediatric Gastroenterology & Nutrition. 44(5):596-602, May 2007.
Objective: To evaluate the efficacy of and adherence to trientine and/or zinc therapy in children with Wilson disease (WD).
Materials and Method: We retrospectively reviewed the clinical records of all children with WD in the paediatric liver/liver transplant program at our institution between 1998 and 2006.
Results: : A total of 22 children with WD were evaluated and treated. Seven with fulminant disease required liver transplantation and 15 were treated with trientine and/or zinc. Ten of those 15 had follow-up for 12 to 60 months and 6 of the latter 10 were followed for 12 to 18 months. All 10 patients were started on a trientine treatment regimen. Mean alanine aminotransferase (ALT) levels decreased from 183 +/- 103 IU at presentation (n = 10) to 80 +/- 46 IU at 12 months (n = 10) and 66 +/- 40 IU at 18 months (n = 7). Mean 24-hour urinary copper levels increased from 156 [mu]g at presentation to 494 [mu]g at 1 to 2 months, then decreased to 71 [mu]g after 21 to 24 months of treatment. Three of 10 patients had normalized ALT levels and 1 patient with cirrhosis continued with normal ALT levels since presentation. Four of 10 patients were documented to be nonadherent, as manifested by increased ALT levels (99 +/- 31 IU); 1 patient had previously normalized ALT levels. In 3 of 10 patients, ALT level decreased but remained at an abnormal level (93 +/- 53 IU).
Conclusion: Trientine and/or zinc therapy is effective for children with WD. Non-adherence is a common cause of increased aminotransferase levels in patients with WD.
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Last Updated On 12/7/2007
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