Abstract

Wilson's disease (WD) is an autosomal recessive inherited disorder of copper (Cu) metabolism. Inflammation is a self-defensive reaction aimed at eliminating or neutralizing injurious stimuli, and restoring tissue integrity. Copper deposition may lead to inflammation in the organs and tissues of WD patients. The aim of this study was to compare the plasma levels of inflammatory cytokines in patients with WD and healthy group, and also to assess whether inflammatory cytokines affects the clinical manifestation of WD. Ninety-nine patients with WD and 32 controls were recruited for this study. Ray Biotech antibody microarray was used to detect the levels of plasma inflammatory cytokines. Our results showed significant increase in T helper (Th) 1 cells (IL-2, TNF-α, and TNF-β), Th2 cells (IL-5, IL-10, and IL-13), and Th17 (IL-23) (p < 0.05). Higher plasma Th 1 cells (IL-2, TNF-α, and TNF-β), Th 2 cells (IL-13), and Th 17 (TGF-β1, IL-23) levels were found in neurological patients compared with control groups (p< 0.01). Besides, we found Th 1 cells (TNF-α and TNF-β), Th 3 (TGF-β1), and Th 17 (IL-23) levels were significantly higher in hepatic and neurological patients (p< 0.05). In addition, the higher Th1 cells (IL-2, TNF-α, and TNF-β), Th2 cells (IL-13), and Th17 (TGF-β1, IL-23) and the course of WD were associated with the severity of the neurological symptoms for WD patients. Altogether, our results indicated that dysregulation of cytokines, mainly increased expression of cytokines and chemokines, occurred in WD patients.

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