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Epidemiology and economic burden of Wilson disease in France: A nationwide population-based study.


ABSTRACT: Wilson disease (WD) is a rare inherited copper metabolism disorder characterized by progressive pathological deposition of copper, primarily in the liver and brain. This longitudinal retrospective study conducted using the French national claims (Système National des Données de Santé [SNDS]) database assessed WD prevalence in France, described patients' characterizations and healthcare resource use and associated costs. Patients with WD were identified from SNDS using the International Classification of Diseases, 10th Revision code E83.0 for copper metabolism disorder or a long-term disease (affection de longue durée [ALD]) associated with this code between 2010 and 2019. Patients were categorized into hepatic, neurological, and psychiatric sub-cohorts. We identified 2287 patients with WD

SUBMITTER: Fang S 

PROVIDER: S-EPMC11670285 | biostudies-literature | 2025 Jan

REPOSITORIES: biostudies-literature