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Guidelines lack data if both variants of 24\u00a0h-UCE measurement (with or without 48\u00a0h of treatment interruption) are equally interpretable.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Eighty-four patients with a confirmed diagnosis of WD treated with chelators (50% of patients with D-Penicillamine and 50% with trientine) and with pairwise 24-h-UCE values on-therapy and off-therapy were included in the analysis. Pairwise urinary sampling between October 2022 (T0) and a 12-month FU (T2) was compared, and exchangeable copper (CuEXC) was additionally measured at T0.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Among the 84 patients, 65% had predominant hepatic symptoms, the median age was 42 years, and 58% were female. At T0, patients were in the stable maintenance phase, with a median treatment duration of 21.9 years. The levels of the biochemical markers liver and copper metabolism remained stable over the 12-month observation period for all patients. 24\u00a0h-UCE off-therapy significantly decreased from T0 to T2 (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.03), whereas no statistically significant differences were detected for 24\u00a0h-UCE after therapy. Both sampling methods did not correlate. CuEXC was significantly correlated with 24\u00a0h-UCE after 48\u00a0h of dose interruption (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.018) but not with 24\u00a0h-UCE after therapy. A total of 46% of the 24\u00a0h-UCE value pairs were discordant, laying out the aimed therapeutic ranges given in current international guidelines.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Off-therapy 24\u00a0h-UCE reflects the \u201cfree\u201d copper pool more accurately than does urinary sampling. The study shows discordant results for both sampling methods in approximately half of the patients, revealing that interpretation of 24\u00a0h-UCE with respect to chelator-dosing decisions should be performed with caution.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13023-025-03545-2","type":"journal-article","created":{"date-parts":[[2025,1,21]],"date-time":"2025-01-21T05:55:00Z","timestamp":1737438900000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["A comparative analysis in monitoring 24-hour urinary copper in wilson disease: sampling on or off treatment?"],"prefix":"10.1186","volume":"20","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7354-0241","authenticated-orcid":false,"given":"Isabelle","family":"Mohr","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Lamade","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christophe","family":"Weber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Viola","family":"Leidner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"K\u00f6hrer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Olkus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Lang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Langel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrischia","family":"Dankert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melanie","family":"Greibich","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silke","family":"Wolf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Holger","family":"Zimmer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Michl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aur\u00e9lia","family":"Poujois","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karl Heinz","family":"Weiss","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uta","family":"Merle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,1,21]]},"reference":[{"issue":"8273","key":"3545_CR1","doi-asserted-by":"publisher","first-page":"643","DOI":"10.1016\/S0140-6736(82)92201-2","volume":"1","author":"JM Walshe","year":"1982","unstructured":"Walshe JM. 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PL, CW, VYL, SK, AO, ML, AL, PD, MG, SW and HZ declare no conflicts of interest. PM received honoraria from Falk, Orphalan and AstraZeneca. AP has received research (institutional) grants from Orphalan, AddMedica and Alexion; consulting fees from Orphalan, Univar, Alexion and Vivet Therapeutics; and speaker fees from Alexion and Orphalan. KHW has received research (institutional) grants from Orphalan; consulting fees from Orphalan, Univar, Pfizer, Alexion and Vivet Therapeutics; speaker fees from Falk, AbbVie, Alexion, and Orphalan; travel assistance to meetings from Alexion and Univar; and advisory board payments from Ultragenyx. UM received honoraria for teaching from Falk and Univar and received travel grants from Gilead and Falk.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"33"}}