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◆ Orphanet Journal of Rare Diseases2026-04-03· Disease

Attenuated dietary motivation in Wilson’s disease correlates with clinical severity

X Wang, Yanxin Wang, Zhao-Hao Ye, Zhilin Zhang, Xun Pan, Xi-Chen Wang, Si-Fan Cen

原始摘要(英文原文)· Original abstract
BACKGROUND & AIMS: Wilson's disease (WD) necessitates lifelong copper-chelation therapy and a restrictive low-copper diet. While the psychological impact of dietary restriction is well-documented in obesity and diabetes, the distinctive eating psychology of WD patients remains unexplored. We aimed to characterize the eating psychology of WD patients and its association with disease severity. METHODS: In a cross-sectional study, 50 WD inpatients and 110 HCs were assessed using the Food Cravings Questionnaire-Trait (FCQ-T) and the Dutch Eating Behavior Questionnaire (DEBQ). WD patients were stratified by liver function, neurological symptoms, and platelet-to-white blood cell ratio (PWR), and prevalent ATP7B genotype (R778L). Group comparisons used non-parametric tests. RESULTS: Compared to HCs, WD patients showed significantly lower scores on all DEBQ dimensions and key FCQ-T facets (all p<0.05). Within the WD cohort, patients with milder liver disease (CTP-A) or no neurological symptoms had significantly higher scores on emotion-driven craving and food-related reinforcement than those with advanced disease (CTP-B/C) or neurological involvement (p<0.05). A low PWR, indicative of severe liver fibrosis, was associated with significantly reduced "Thoughts about food" (U=396.5, p=0.028). Exploratory analysis based on the R778L mutation revealed no significant association between genotype and dietary psychological scores. CONCLUSIONS: WD is associated with a generally attenuated dietary motivation; however, patients with less severe disease exhibit a characteristic psychological profile with relatively stronger reward-related eating motivations compared to their more severely affected counterparts. These findings highlight the need for integrated psychometric assessment in WD management to guide personalized dietary interventions and improve outcomes.
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