{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T08:55:29Z","timestamp":1790412929824,"version":"4.1.0"},"reference-count":23,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2013,12,7]],"date-time":"2013-12-07T00:00:00Z","timestamp":1386374400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2014,2]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Wilson's disease (<jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content>) is an inherited copper metabolism disorder that leads to dysfunction of affected tissues, mostly in the liver and brain. Anti\u2010copper treatment should prevent clinically overt <jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content> in pre\u2010symptomatic patients but this has not been supported by strong evidence. Our aim was to evaluate the long\u2010term effectiveness of treatment in clinically pre\u2010symptomatic patients, with particular emphasis on patient compliance with treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data were analyzed for 87 consecutive patients with no clinical symptoms of <jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content> who were identified between 1957 and 2009 by family screening. All of them since diagnosis were treated with either zinc sulphate (<jats:styled-content style=\"fixed-case\">Z<\/jats:styled-content>n) (66.7%) or <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>\u2010penicillamine (<jats:styled-content style=\"fixed-case\">DPA<\/jats:styled-content>) (33.3%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>During a median follow\u2010up of 12\u00a0years (range 3\u201352), 55 (63%) patients remained without clinical symptoms, 13 (15%) developed neuropsychiatric symptoms and 21 (24%) developed hepatic dysfunction, including five deaths from hepatic failure. Non\u2010compliance for at least three consecutive months was observed in 39 patients, and in 29 cases this extended for more than 12\u00a0months. Multivariate analysis showed that the odds of developing symptomatic <jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content> were independently increased by non\u2010compliance (odds ratio 24.0, 95% confidence interval 6.0\u201399.0). According to <jats:styled-content style=\"fixed-case\">K<\/jats:styled-content>aplan\u2013<jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>eier analysis patients who were compliant to treatment had a significantly higher likelihood of remaining symptom\u2010free, and their overall survival was similar to the survival rate observed in the general population.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The use of anti\u2010copper agents in clinically pre\u2010symptomatic patients diagnosed with <jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content> allows clinically overt disease to be effectively prevented. However, compliance with therapy is extremely important.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.12320","type":"journal-article","created":{"date-parts":[[2013,12,7]],"date-time":"2013-12-07T05:51:56Z","timestamp":1386395516000},"page":"332-337","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":84,"title":["Compliant treatment with anti\u2010copper agents prevents clinically overt <scp>W<\/scp>ilson's disease in pre\u2010symptomatic patients"],"prefix":"10.1111","volume":"21","author":[{"given":"K.","family":"Dzie\u017cyc","sequence":"first","affiliation":[{"name":"Second Department of Neurology Institute of Psychiatry and Neurology Warsaw Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Karli\u0144ski","sequence":"additional","affiliation":[{"name":"Second Department of Neurology Institute of Psychiatry and Neurology Warsaw 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