Honghao Wang1, Nan Cheng2, Jianjian Dong3, Xun Wang1, Yongsheng Han3, Renmin Yang3, Yongzhu Han4. 1. Department of Neurology, Nanfang Hospital, Southern Medical University, Guangzhou, China. 2. Hospital affiliated to Institute of Neurology, Anhui College of TCM, Hefei, China; Hefei Institute of Physical Science, CAS, China. 3. Hospital affiliated to Institute of Neurology, Anhui College of TCM, Hefei, China. 4. Hospital affiliated to Institute of Neurology, Anhui College of TCM, Hefei, China. Electronic address: hanyongzhutcm@163.com.
Abstract
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.
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 WDpatients. Pentraxin 3 (PTX3) may play an important role in innate immunity and in WD. We compared plasma PTX3 concentrations in WDpatients and healthy controls, and to determine whether PTX3 concentration was associated with neurological disease severity. METHODS: This study included 86 WDpatients 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 WDpatients 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.