Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry
Background and Aims Monitoring Wilson disease (WD) is challenging due to its variable presentation and the absence of reliable biomarkers. This study aims to assess the predictive value of liver enzymes, particularly transaminases, on long-term outcomes in patients with hepatic WD using data from th...
| Autores: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Tipo de recurso: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2025 |
| País: | España |
| Institución: | Universidad de Barcelona |
| Repositorio: | Dipòsit Digital de la UB |
| OAI Identifier: | oai:diposit.ub.edu:2445/222286 |
| Acceso en línea: | https://hdl.handle.net/2445/222286 |
| Access Level: | acceso abierto |
| Palabra clave: | Malalties del fetge Marcadors bioquímics Liver diseases Biochemical markers |
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Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registryBerenguer, MarinaGarcía Villarreal, LuisOlveira, AntonioMollina Pérez, EstherMoreno Planas, José MaríaRomero Gutiérrez, MartaPinazo Bandera, José MaríaMasnou Ridaura, HelenaIruzubieta, PaulaGonzález Diéguez, María LuisaAmpuero, JavierFernández Ramos, José RamónMuñoz, CarolinaArencibia Almeida, AnaLorente, SaraDelgado Blanco, ManuelBurgos Santamaría, DiegoPons Delgado, MònicaCachero, AlbaHernández Guerra, ManuelGómez Camarero, JudithGil Rojas, SergioLázaro Ríos, MaríaCarmona Soria, IsabelCarrión, GemmaBono, AriadnaMiralpeix, AnnaAlonso Castellano, PabloMariño, ZoeRegistro Wilson-AeehMalalties del fetgeMarcadors bioquímicsLiver diseasesBiochemical markersBackground and Aims Monitoring Wilson disease (WD) is challenging due to its variable presentation and the absence of reliable biomarkers. This study aims to assess the predictive value of liver enzymes, particularly transaminases, on long-term outcomes in patients with hepatic WD using data from the Spanish Wilson Registry. Patients and Methods We analysed data from 162 WD patients with hepatic involvement and over one year of follow-up. Patients were classified as mild (no cirrhosis) or severe (with cirrhosis) at diagnosis. An unstable pattern of transaminases was defined as recurrent AST or ALT elevations. Unfavourable outcomes included new cirrhosis, elastography progression > 2 Kpa, liver transplant, or liver-related deaths. Logistic regression models were used to evaluate the impact of various factors on disease outcome. Results Of 162 patients, 81.5% had mild disease at diagnosis. Most received chelators as first-line therapy, achieving an 81.4% one-year biochemical response. After a median follow-up of 17 years, 59% exhibited an unstable transaminase pattern, and 29% had an unfavourable outcome. Key factors associated with poor outcome included older age at diagnosis (OR = 1.03), lack of early biochemical response (OR = 0.19), advanced disease markers (platelet count, albumin), and an unstable transaminase pattern (OR = 2.92). Transaminase levels did not predict outcomes based on initial disease severity. Even patients with mild disease at diagnosis and persistently normal transaminases could experience progression over time, underscoring the need for more thorough follow-up evaluations. Conclusion While transaminases are valuable for monitoring WD, they should be used alongside other biomarkers to better predict disease progression.Springer Science and Business Media LLC2025info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/2445/222286Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))reponame:Dipòsit Digital de la UBinstname:Universidad de BarcelonaInglésReproducció del document publicat a: https://doi.org/10.1186/s13023-025-03821-1Orphanet Journal of Rare Diseases, 2025, vol. 20, num. 1https://doi.org/10.1186/s13023-025-03821-1cc by (c) Berenguer, Marina et al, 2025http://creativecommons.org/licenses/by/3.0/es/info:eu-repo/semantics/openAccessoai:diposit.ub.edu:2445/2222862026-05-27T06:46:51Z |
| dc.title.none.fl_str_mv |
Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry |
| title |
Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry |
| spellingShingle |
Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry Berenguer, Marina Malalties del fetge Marcadors bioquímics Liver diseases Biochemical markers |
| title_short |
Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry |
| title_full |
Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry |
| title_fullStr |
Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry |
| title_full_unstemmed |
Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry |
| title_sort |
Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry |
| dc.creator.none.fl_str_mv |
Berenguer, Marina García Villarreal, Luis Olveira, Antonio Mollina Pérez, Esther Moreno Planas, José María Romero Gutiérrez, Marta Pinazo Bandera, José María Masnou Ridaura, Helena Iruzubieta, Paula González Diéguez, María Luisa Ampuero, Javier Fernández Ramos, José Ramón Muñoz, Carolina Arencibia Almeida, Ana Lorente, Sara Delgado Blanco, Manuel Burgos Santamaría, Diego Pons Delgado, Mònica Cachero, Alba Hernández Guerra, Manuel Gómez Camarero, Judith Gil Rojas, Sergio Lázaro Ríos, María Carmona Soria, Isabel Carrión, Gemma Bono, Ariadna Miralpeix, Anna Alonso Castellano, Pablo Mariño, Zoe Registro Wilson-Aeeh |
| author |
Berenguer, Marina |
| author_facet |
Berenguer, Marina García Villarreal, Luis Olveira, Antonio Mollina Pérez, Esther Moreno Planas, José María Romero Gutiérrez, Marta Pinazo Bandera, José María Masnou Ridaura, Helena Iruzubieta, Paula González Diéguez, María Luisa Ampuero, Javier Fernández Ramos, José Ramón Muñoz, Carolina Arencibia Almeida, Ana Lorente, Sara Delgado Blanco, Manuel Burgos Santamaría, Diego Pons Delgado, Mònica Cachero, Alba Hernández Guerra, Manuel Gómez Camarero, Judith Gil Rojas, Sergio Lázaro Ríos, María Carmona Soria, Isabel Carrión, Gemma Bono, Ariadna Miralpeix, Anna Alonso Castellano, Pablo Mariño, Zoe Registro Wilson-Aeeh |
| author_role |
author |
| author2 |
García Villarreal, Luis Olveira, Antonio Mollina Pérez, Esther Moreno Planas, José María Romero Gutiérrez, Marta Pinazo Bandera, José María Masnou Ridaura, Helena Iruzubieta, Paula González Diéguez, María Luisa Ampuero, Javier Fernández Ramos, José Ramón Muñoz, Carolina Arencibia Almeida, Ana Lorente, Sara Delgado Blanco, Manuel Burgos Santamaría, Diego Pons Delgado, Mònica Cachero, Alba Hernández Guerra, Manuel Gómez Camarero, Judith Gil Rojas, Sergio Lázaro Ríos, María Carmona Soria, Isabel Carrión, Gemma Bono, Ariadna Miralpeix, Anna Alonso Castellano, Pablo Mariño, Zoe Registro Wilson-Aeeh |
| author2_role |
author author author author author author author author author author author author author author author author author author author author author author author author author author author author author |
| dc.subject.none.fl_str_mv |
Malalties del fetge Marcadors bioquímics Liver diseases Biochemical markers |
| topic |
Malalties del fetge Marcadors bioquímics Liver diseases Biochemical markers |
| description |
Background and Aims Monitoring Wilson disease (WD) is challenging due to its variable presentation and the absence of reliable biomarkers. This study aims to assess the predictive value of liver enzymes, particularly transaminases, on long-term outcomes in patients with hepatic WD using data from the Spanish Wilson Registry. Patients and Methods We analysed data from 162 WD patients with hepatic involvement and over one year of follow-up. Patients were classified as mild (no cirrhosis) or severe (with cirrhosis) at diagnosis. An unstable pattern of transaminases was defined as recurrent AST or ALT elevations. Unfavourable outcomes included new cirrhosis, elastography progression > 2 Kpa, liver transplant, or liver-related deaths. Logistic regression models were used to evaluate the impact of various factors on disease outcome. Results Of 162 patients, 81.5% had mild disease at diagnosis. Most received chelators as first-line therapy, achieving an 81.4% one-year biochemical response. After a median follow-up of 17 years, 59% exhibited an unstable transaminase pattern, and 29% had an unfavourable outcome. Key factors associated with poor outcome included older age at diagnosis (OR = 1.03), lack of early biochemical response (OR = 0.19), advanced disease markers (platelet count, albumin), and an unstable transaminase pattern (OR = 2.92). Transaminase levels did not predict outcomes based on initial disease severity. Even patients with mild disease at diagnosis and persistently normal transaminases could experience progression over time, underscoring the need for more thorough follow-up evaluations. Conclusion While transaminases are valuable for monitoring WD, they should be used alongside other biomarkers to better predict disease progression. |
| publishDate |
2025 |
| dc.date.none.fl_str_mv |
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/2445/222286 |
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https://hdl.handle.net/2445/222286 |
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Inglés |
| language_invalid_str_mv |
Inglés |
| dc.relation.none.fl_str_mv |
Reproducció del document publicat a: https://doi.org/10.1186/s13023-025-03821-1 Orphanet Journal of Rare Diseases, 2025, vol. 20, num. 1 https://doi.org/10.1186/s13023-025-03821-1 |
| dc.rights.none.fl_str_mv |
cc by (c) Berenguer, Marina et al, 2025 http://creativecommons.org/licenses/by/3.0/es/ info:eu-repo/semantics/openAccess |
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cc by (c) Berenguer, Marina et al, 2025 http://creativecommons.org/licenses/by/3.0/es/ |
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openAccess |
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application/pdf |
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Springer Science and Business Media LLC |
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Springer Science and Business Media LLC |
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Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) reponame:Dipòsit Digital de la UB instname:Universidad de Barcelona |
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Universidad de Barcelona |
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Dipòsit Digital de la UB |
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Dipòsit Digital de la UB |
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