Research On Iron Metabolism And Cognitive Dysfunction Of Patients With Wilson Disease And Their Related Serum Mar Kers

ObjectsTo analyze the correlations between the results of iron metabolism and the score of MMSE of WD patients and their relationships with the levels of?-synuclein, Tau and A? in serum by testing the index of iron metabolism, the score of MMSE and the level of ?-Synuclein, Tau and A? in serum of patients with WD, HBV and normal controls, and explore whether cognitive dysfunction in patients with WD is associated with abnormal iron metabolism.Methods46 cases of WD patients, who were admitted to the Affiliated Hospital of Neurology Institute, Anhui University of Chinese medicine, were collected as experimental group, consisting of 22 hepatic WD patients and 24 cerebral WD patients. And 21 HBV patients and 17 healthy people were considered as control groups. The index of iron metabolism, copper metabolism and the level of?-synuclein, Tau and A? in the peripheral blood of them were detected.Meanwhile, MMSE was used to measure their intelligence, and their name, sex,age, course of the disease, first symptom and other clinical data were recorded.Then the correlation between iron metabolism, cognitive dysfunction and related biomarkers of these groups were analyzed by statistical software.Results1. Comparison of iron metabolism in each groupCompared with normal group, the SI was reduced in experience group(p <0.05), the patients of hepatic WD had an elevated SF content(p < 0.05) and the level of TF in experience group was significantly reduced(p < 0.01). However,compared with B-hepatitis group, both experience group and normal group all hada significantly reduced level of SI(p < 0.01) and a higher TIBC(p < 0.05), and the content of SF all were reduced in patients with cerebral WD(p < 0.01) and in patients of hepatic WD(p < 0.05). Meanwhile there was an increased TF(p < 0.01)but a reduced STfR(p < 0.05) in experience group compared with HBV group.Compared with patients of cerebral WD, patients of hepatic WD had an elevated level of STfR(p < 0.05).2. Comparison of the score of MMSE in each groupAll of the normal group, HBV group and patients of hepatic WD had a significantly higher(p < 0.01) MMSE score than the cerebral WD.3. Comparison of the index of serum marker in each groupCompared with normal group, there were an increased A?(p < 0.01) and Tau(p < 0.05) in the patients of cerebral WD. And both the experience group and normal group had significantly increased content of ?-synuclein, Tau in serum(p< 0.01) than the HBV group.4. Comparison of the index of copper metabolism in each groupCompared with normal group, the serum Cu and CP decreased significantly(p < 0.01) in WD patients, and in HBV group there was an increased content of Cu(p < 0.05). The serum Cu and CP were significantly reduced(p < 0.01) in WD patients compared with HBV group.5. The correlation between the disorder of iron metabolism and the score of MMSE in cerebral WD5.1The SI, SF, TF and STfR all were positively correlated with the score of MMSE in cerebral WD.5.2 The TIBC was negatively correlated with the score of MMSE in cerebral WD.6. The correlation between the disorder of iron metabolism and the level of serum marker in cerebral WDThe SI, SF, TF and STfR were negatively correlated with ?-synuclein, Tau and A?(p < 0.01) in the serum of cerebral WD.7. The correlation between the score of MMSE and the level of serum marker in cerebral WDThe ?-synuclein, Tau, and A? in serum were negatively correlated with the score of MMSE in cerebral WD(p < 0.01).8. The correlation between the disorder of iron metabolism and the level of copper metabolism in cerebral WDThe SI, SF, TF and STfR were positively correlated with CP and Cu in the serum of cerebral WD(p < 0.01).Conclusions1. It has been showed that all the patients with cerebral WD and hepaticWD enrolled in this study have different degrees of iron metabolism abnormality.2. The cognitive dysfunction of patients with cerebral WD may be consisted with the changes of ?-synuclein, Tau and A? in serum.3. Abnormal iron metabolism may be one of main reasons of the cognitive dysfunction of patients with cerebral WD through participation in cognitive impairment process.