{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T13:28:40Z","timestamp":1780493320988,"version":"3.54.1"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,2,11]],"date-time":"2019-02-11T00:00:00Z","timestamp":1549843200000},"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":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2019,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>Urinary copper excretion rates and non\u2010caeruloplasmin associated copper concentrations are increased in patients with Wilson disease. However, there is little literature describing the monitoring of these parameters over the long term.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This is a monocentric retrospective study including data collected between 2003 and 2015 from 321 patients with Wilson disease by chart review. The patients were under therapy with D\u2010penicillamine, trientine, or zinc. 24\u2010h urinary copper excretion rates, non\u2010caeruloplasmin associated copper, and total serum copper concentrations were determined at the start of therapy, as well as 6, 12, 18, 24, 36, and \u2265 60 months after the start of therapy. For patients taking chelating agents, all parameters were measured while under continued therapy, as well as after a 48\u2010h dose interruption. A mathematical formula to predict 24\u2010h urinary copper excretion rates under different therapies was established.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In all treatment groups, urinary copper excretion rates decreased over time, but the inter\u2010individual variation of the results was high. Non\u2010caeruloplasmin associated copper concentrations tended to decline over time, but with a higher variation of results than that observed for urinary copper excretion rates.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Due to their variability, urinary copper excretion rates and serum copper concentrations are less than ideal parameters by which to monitor the benefit of a copper\u2010reducing therapy. Urinary copper excretion rates seem to be more suitable than non\u2010caeruloplasmin associated copper concentrations for this purpose.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jimd.12046","type":"journal-article","created":{"date-parts":[[2019,2,12]],"date-time":"2019-02-12T05:41:37Z","timestamp":1549950097000},"page":"371-380","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":37,"title":["Long\u2010term evaluation of urinary copper excretion and non\u2010caeruloplasmin associated copper in Wilson disease patients under medical treatment"],"prefix":"10.1002","volume":"42","author":[{"given":"Jan","family":"Pfeiffenberger","sequence":"first","affiliation":[{"name":"Department of Gastroenterology and Hepatology University Hospital Heidelberg  INF 410, 69120 Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine Marie","family":"Lohse","sequence":"additional","affiliation":[{"name":"Department 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