Background: Wilson's disease (WD) is an autosomal recessive inherited disorder of copper (Cu) metabolism, resulting in pathological accumulation of Cu in many organs and tissues, predominantly in the liver and brain. Cu deposition may lead to neuroinflammation in the brain of WD patients. Pentraxin 3 (PTX3) may play an important role in innate immunity and in WD. We compared plasma PTX3 concentrations in WD patients and healthy controls, and to determine whether PTX3 concentration was associated with neurological disease severity.
Methods: This study included 86 WD patients and 28 controls. Plasma PTX3 and C-reactive protein (CRP) concentration levels were measured using specific enzyme-linked immunosorbent assays. Disease severity was determined using the neurological Global Assessment Scale (GAS) for WD.
Results: Plasma PTX3 levels were significantly higher in patients with neurological WD than in controls. PTX3 levels in WD patients were associated with neurological disease severity. However, there was no correlation between CRP and neurological GAS scores.
Conclusions: PTX3 represents a potential biochemical marker of disease severity in patients with neurological WD.
Keywords: Biochemical marker; Neuroinflammation; Pentraxin 3; Wilson's disease.
Copyright © 2016. Published by Elsevier B.V.