Exchangeable copper for patients with Wilson disease at follow-up: Rethinking normal ranges or changing methodology

Background and aim: Determining suitable copper parameters for monitoring Wilson disease remains a topic of ongoing discussion. International recommendations currently rely on the combination of urinary copper excretion and nonspecific liver markers when considering therapy and time elapsed since di...

ver descrição completa

Detalhes bibliográficos
Autores: Mariño Méndez, Zoe, García Solà, Clàudia, Ríos, José, Bono, Ariadna, García, Sonia, Miralpeix, Anna, Andreu, Rocío, Aguado, Cristina, Forns, Xavier, Torra, Mercè, Berenguer, Marina
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2024
País:España
Recursos:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositorio:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:2445/217323
Acesso em linha:https://hdl.handle.net/2445/217323
Access Level:acceso abierto
Palavra-chave:Intoxicació plúmbica
Coure en l'organisme
Homeòstasi
Metodologia
Lead poisoning
Copper in the body
Homeostasis
Methodology
id ES_2a9804ebfd91b2a4c65be6e3a96a7fd7
oai_identifier_str oai:recercat.cat:2445/217323
network_acronym_str ES
network_name_str España
repository_id_str
spelling Exchangeable copper for patients with Wilson disease at follow-up: Rethinking normal ranges or changing methodologyMariño Méndez, ZoeGarcía Solà, ClàudiaRíos, JoséBono, AriadnaGarcía, SoniaMiralpeix, AnnaAndreu, RocíoAguado, CristinaForns, XavierTorra, MercèBerenguer, MarinaIntoxicació plúmbicaCoure en l'organismeHomeòstasiMetodologiaLead poisoningCopper in the bodyHomeostasisMethodologyBackground and aim: Determining suitable copper parameters for monitoring Wilson disease remains a topic of ongoing discussion. International recommendations currently rely on the combination of urinary copper excretion and nonspecific liver markers when considering therapy and time elapsed since diagnosis. The emergence of exchangeable copper (CuEX) as a novel measurement reflecting the "free copper pool" held promise as a valuable target to ensure metabolic stability during follow-up, although the validation of target ranges remains unknown. We aimed to evaluate CuEX quantification in repeated samples from 92 real-world patients with Wilson disease during a 2-year period. Approach: Patients were classified as "stable" if a diagnosis had been made more than 1 year before and were compliant with stable anti-copper drug and dose. Otherwise, patients were classified as "nonstable." Results: Two hundred and thirteen CuEX samples were obtained per clinical practice. Overall, 57% of CuEX measurements fell below the reference "range of normality," whereas only 34% were within and 9% were above normal levels. There was no association of CuEX levels with therapy, elapsed time from diagnosis, or clinical stability, although most of the samples above normality corresponded to nonstable patients. Only 23.4% of the CuEX samples were aligned with data obtained from concomitant urinary copper excretion. Conclusions: Our findings suggest that CuEX is a suboptimal tool for assessing copper homeostasis when used alone and should be used with caution if no additional information is available. Normal reference intervals for Wilson disease-treated patients should be redefined, as most CuEX quantifications fell in the lower range, with no sign of overtreatment in these patients.Wiley2025202520242025info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion31 p.application/pdfhttps://hdl.handle.net/2445/217323Articles publicats en revistes (Medicina)reponame:Recercat. Dipósit de la Recerca de Catalunyainstname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)InglésReproducció del document publicat a: https://doi.org/10.1097/HEP.0000000000001105Hepatology, 2024https://doi.org/10.1097/HEP.0000000000001105cc-by-nc (c) Mariño Méndez, Zoe et al., 2024http://creativecommons.org/licenses/by-nc/4.0/info:eu-repo/semantics/openAccessoai:recercat.cat:2445/2173232026-05-29T05:05:01Z
dc.title.none.fl_str_mv Exchangeable copper for patients with Wilson disease at follow-up: Rethinking normal ranges or changing methodology
title Exchangeable copper for patients with Wilson disease at follow-up: Rethinking normal ranges or changing methodology
spellingShingle Exchangeable copper for patients with Wilson disease at follow-up: Rethinking normal ranges or changing methodology
Mariño Méndez, Zoe
Intoxicació plúmbica
Coure en l'organisme
Homeòstasi
Metodologia
Lead poisoning
Copper in the body
Homeostasis
Methodology
title_short Exchangeable copper for patients with Wilson disease at follow-up: Rethinking normal ranges or changing methodology
title_full Exchangeable copper for patients with Wilson disease at follow-up: Rethinking normal ranges or changing methodology
title_fullStr Exchangeable copper for patients with Wilson disease at follow-up: Rethinking normal ranges or changing methodology
title_full_unstemmed Exchangeable copper for patients with Wilson disease at follow-up: Rethinking normal ranges or changing methodology
title_sort Exchangeable copper for patients with Wilson disease at follow-up: Rethinking normal ranges or changing methodology
dc.creator.none.fl_str_mv Mariño Méndez, Zoe
García Solà, Clàudia
Ríos, José
Bono, Ariadna
García, Sonia
Miralpeix, Anna
Andreu, Rocío
Aguado, Cristina
Forns, Xavier
Torra, Mercè
Berenguer, Marina
author Mariño Méndez, Zoe
author_facet Mariño Méndez, Zoe
García Solà, Clàudia
Ríos, José
Bono, Ariadna
García, Sonia
Miralpeix, Anna
Andreu, Rocío
Aguado, Cristina
Forns, Xavier
Torra, Mercè
Berenguer, Marina
author_role author
author2 García Solà, Clàudia
Ríos, José
Bono, Ariadna
García, Sonia
Miralpeix, Anna
Andreu, Rocío
Aguado, Cristina
Forns, Xavier
Torra, Mercè
Berenguer, Marina
author2_role author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Intoxicació plúmbica
Coure en l'organisme
Homeòstasi
Metodologia
Lead poisoning
Copper in the body
Homeostasis
Methodology
topic Intoxicació plúmbica
Coure en l'organisme
Homeòstasi
Metodologia
Lead poisoning
Copper in the body
Homeostasis
Methodology
description Background and aim: Determining suitable copper parameters for monitoring Wilson disease remains a topic of ongoing discussion. International recommendations currently rely on the combination of urinary copper excretion and nonspecific liver markers when considering therapy and time elapsed since diagnosis. The emergence of exchangeable copper (CuEX) as a novel measurement reflecting the "free copper pool" held promise as a valuable target to ensure metabolic stability during follow-up, although the validation of target ranges remains unknown. We aimed to evaluate CuEX quantification in repeated samples from 92 real-world patients with Wilson disease during a 2-year period. Approach: Patients were classified as "stable" if a diagnosis had been made more than 1 year before and were compliant with stable anti-copper drug and dose. Otherwise, patients were classified as "nonstable." Results: Two hundred and thirteen CuEX samples were obtained per clinical practice. Overall, 57% of CuEX measurements fell below the reference "range of normality," whereas only 34% were within and 9% were above normal levels. There was no association of CuEX levels with therapy, elapsed time from diagnosis, or clinical stability, although most of the samples above normality corresponded to nonstable patients. Only 23.4% of the CuEX samples were aligned with data obtained from concomitant urinary copper excretion. Conclusions: Our findings suggest that CuEX is a suboptimal tool for assessing copper homeostasis when used alone and should be used with caution if no additional information is available. Normal reference intervals for Wilson disease-treated patients should be redefined, as most CuEX quantifications fell in the lower range, with no sign of overtreatment in these patients.
publishDate 2024
dc.date.none.fl_str_mv 2024
2025
2025
2025
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/2445/217323
url https://hdl.handle.net/2445/217323
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Reproducció del document publicat a: https://doi.org/10.1097/HEP.0000000000001105
Hepatology, 2024
https://doi.org/10.1097/HEP.0000000000001105
dc.rights.none.fl_str_mv cc-by-nc (c) Mariño Méndez, Zoe et al., 2024
http://creativecommons.org/licenses/by-nc/4.0/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv cc-by-nc (c) Mariño Méndez, Zoe et al., 2024
http://creativecommons.org/licenses/by-nc/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv 31 p.
application/pdf
dc.publisher.none.fl_str_mv Wiley
publisher.none.fl_str_mv Wiley
dc.source.none.fl_str_mv Articles publicats en revistes (Medicina)
reponame:Recercat. Dipósit de la Recerca de Catalunya
instname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
instname_str Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
reponame_str Recercat. Dipósit de la Recerca de Catalunya
collection Recercat. Dipósit de la Recerca de Catalunya
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869405082633633792
score 15,812429

Registos relacionados