Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry
Year: 2025
Type: article
Study design: Observational Study
Abstract: 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.
Authors: Marina Berenguer, Luis García-Villarreal, Antonio Olveira, Esther Mollina Pérez, José María Moreno Planas, Marta Romero-Gutiérrez, José María Pinazo Bandera, Helena Masnou Ridaura, Paula Iruzubieta, María Luisa González Diéguez, Javier Ampuero, José Ramón Fernández Ramos, Carolina Muñoz, Ana Arencibia Almeida, Sara Lorente, Manuel Delgado Blanco, Diego Burgos Santamaría, Mònica Pons Delgado, Alba Cachero, Manuel Hernández Guerra, Judith Gómez Camarero, Sergio Gil Rojas, María Lázaro Ríos, Isabel Carmona Soria, Gemma Carrión, Ariadna Bono, Anna Miralpeix, Pablo Alonso Castellano, Zoe Mariño, Registro Wilson-AEEH
Institutions: Universitat de València, Hospital Universitari i Politècnic La Fe, Universidad de Las Palmas de Gran Canaria, Hospital Universitario La Paz, Complejo Hospitalario Universitario de Albacete, University of Castilla-La Mancha, Hospital Universitario de Toledo, Hospital Clínico Universitario Virgen de la Victoria, Instituto de Investigación Biomédica de Málaga, Marqués de Valdecilla University Hospital, Hospital Universitario Central de Asturias, Hospital Universitario Virgen del Rocío, Hospital de Cruces, Hospital Universitario 12 De Octubre, Hospital Universitario Nuestra Señora de Candelaria, Hospital Clínico Universitario Lozano Blesa, Complexo Hospitalario Universitario A Coruña, Hospital Universitario Ramón y Cajal, Universitat Autònoma de Barcelona, Vall d'Hebron Hospital Universitari, Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas, Bellvitge University Hospital, Hospital Universitario de Canarias, Hospital Universitario de Burgos, Hospital Virgen de la Luz, Hospital Universitario Miguel Servet, Hospital Universitario Virgen Macarena, Hospital Universitario Infanta Leonor, Consorci Institut D'Investigacions Biomediques August Pi I Sunyer, Universitat de Barcelona
Language: English
Topic: Trace Elements in Health
Subfield: Nutrition and Dietetics
Field: Nursing
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,990
OpenAlex ID: W4411118963