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Objective To evaluate the clinical manifestations, metal metabolism, imaging characteristics and treatment response in patients with delayed Wilson disease (WD). Methods Patients with untreated WD (40 with delayed onset and 40 with non?delayed onset) were enrolled. Twenty healthy people were included as normal controls. All patients were evaluated with modified Young scale neural symptom scores, grade of Child liver function and mental symptoms rating scale, magnetic resonance imaging (MRI) scan, magnetic sensitive imaging (susceptibility weighted imaging, SWI), metal metabolism. Corrected phase (CP) was measured at SWI. After 2 week treatment, neurologic symptoms, liver function, and metal metabolism were reviewed. Results The total score of neurological symptoms in WD patients with delayed onset was lower than that of non?delayed onset (13.00 ± 6.87 vs. 21.13 ± 5.53, P=0.033). The scores of SCL?90 and HAMA depression scales in patients with delayed onset were lower than those of non?delayed onset. On T2 weighted imaging, areas including substantia nigra and thalamus, the caudate nucleus, globus pallidus, putamen presented high signal rate in patients with delated onset than those with non?delayed (P=0.022, 0.037, 0.022, 0.037, 0.029 respectively). The SWI CP values of cangbai sphere and shell nucleus in patients with delayed onset were lower than those with non?delayed onset. Patients with delayed onset had higher urinary copper than those with non?delayed onset before and after treatment (P=0.040, 0.036). After treatment, the score of abnormal tremor and gait in patients with delayed onset was decreased (P=0.037, 0.044), while as the occurrence of neurological symptoms was increased by 10%, and the liver function level in patients with delayed WD was decreased in 3 cases. Conclusions The brain of WD patients with delayed onset is mainly composed of metal deposits, however the cell damage is not apparent. Clinical symptoms are characterized by significant liver injury, but relatively mild neurological and psychiatric symptoms. Patients with delayed WD have higher urinary copper excretion than those with non?delayed WD. Chelating agents improves the neurological symptoms in patients with delayed onset.
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Objective To investigate the value of susceptibility weighted imaging (SWI) with T1ρimaging in staging of hepatic fibrosis(HF) in rabbits. Methods Eighty selected white rabbits from New Zealand were randomly divided into the HF group (n=60) and the control group (n=20). Rabbits in the HF group were injected subcutaneously with 50％CCl4 oil solution to establish HF model,and the normal control rabbits were injected with saline solution subcutaneously.The HF group(n=15) and control group(n=5) were randomly selected at the 4th, 5th, 6th and 10th week after injection, to undergo liver MR scan. The liver signal intensity (SI liver), the muscle signal intensity (SI muscle),liver-to-muscle SI ratios (SIR) and liver T1ρvalues were measured. Scheuer was adopted to stage the rabbits in HF. One-way analysis of variance was used to compare the differences between SIR and T1ρ values in different stages of HF pathological. Spearman correlation was used to analyze the correlation between SIR and T1ρ values in the different stage of HF pathological.The ROC were used to compare the efficacy between SIR and T1ρvalues in the diagnosis of HF pathological stage. Results Among the final qualified 68 rabbits in the study, 17 in F0 phase, 11 in F1 phase, 16 in F2 phase, 11 in F3 phase, and 13 in F4 phase. The SIR were (0.977 ± 0.013), (0.960 ± 0.015), (0.802 ± 0.026), (0.786 ± 0.022), (0.541 ± 0.116); T1ρ values were (22.301 ± 1.849), (24.034 ± 0.867), (25.374 ± 1.309),(25.364±1.945),(30.948±2.925) ms.There were statistically significant in SIR between F0 and F2,F0 and F3, F0 and F4, F1 and F2, F1 and F3, F1 and F4, F2 and F4, F3 and F4 (P<0.01). There were statistically significant in T1ρvalues between F0 and F1,F0 and F2,F0 and F3,F0 and F4,F1 and F2,F1 and F4,F2 and F4, F3 and F4(P<0.05). SIR were negatively correlated with HF staging while T1ρ values were positively. ROC showed that the AUC of the T1ρvalues was slightly larger than SIR in the F4 group(0.992>0.966),and the AUC of the SIR was greater than T1ρvalues in the other groups. Conclusion SWI and T1ρvalues can provide an important objective basis in staging of HF. Both of them have great clinical application prospects but SIR diagnostic efficiency is slightly better than that of T1ρvalues.
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Objective:To analyze the imaging features of magnetic susceptibility-weighted imaging (SWI) in the patients with cerebral amyloid angiopathy-related hemorrhage (CAAH),and to clarify the diagnostic value of SWI for CAAH.Methods:A total of 68 patients presumptively diagnosed as CAAH were collected,and their imaging data of routine MRI and SWI were collected and analyzed.The detection rates of hemorrhage focus of the CAAH patients were compared between two kinds of imaging examination.The consistency of detection of CAAH by routine MRI and SWI was analyzed.The imaging features of SWI and the risk of focus hemorrhage in the patients with CAAH were analyzed by multivariate Logistic regression analysis.Results:Sixty-one patients were confirmed as CAAH by pathologic diagnosis,and 53 patients were confirmed as CAAH by routine MRI;the detection rate was 86.89％;59 cases of hemorrhage focus were confirmed by SWI and the detection rate was 96.72％.The number of lesions detected by SWI was more than that of routine MRI (P＜0.05).The consistency of detection of CAAH by routine MRI and SWI was poor,and the value of Kappa was 0.3666.The patchy high signal and multiple clear edge low signal area were the relative imaging features of CAAH with SWI in the patients with CAAH analyzed by multivariate Logistic regression analysis (OR=3.895,P=0.025;OR=3.124,P=0.029).Conclusion:SWI can efficiently detect the hemorrhage focus in the patients with CAAH and the diagnostic value is better than routine MRI.
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Objective:To analyze the imaging features of magnetic susceptibility-weighted imaging (SWI) in the patients with cerebral amyloid angiopathy-related hemorrhage (CAAH),and to clarify the diagnostic value of SWI for CAAH.Methods:A total of 68 patients presumptively diagnosed as CAAH were collected,and their imaging data of routine MRI and SWI were collected and analyzed.The detection rates of hemorrhage focus of the CAAH patients were compared between two kinds of imaging examination.The consistency of detection of CAAH by routine MRI and SWI was analyzed.The imaging features of SWI and the risk of focus hemorrhage in the patients with CAAH were analyzed by multivariate Logistic regression analysis.Results:Sixty-one patients were confirmed as CAAH by pathologic diagnosis,and 53 patients were confirmed as CAAH by routine MRI;the detection rate was 86.89％;59 cases of hemorrhage focus were confirmed by SWI and the detection rate was 96.72％.The number of lesions detected by SWI was more than that of routine MRI (P＜0.05).The consistency of detection of CAAH by routine MRI and SWI was poor,and the value of Kappa was 0.3666.The patchy high signal and multiple clear edge low signal area were the relative imaging features of CAAH with SWI in the patients with CAAH analyzed by multivariate Logistic regression analysis (OR=3.895,P=0.025;OR=3.124,P=0.029).Conclusion:SWI can efficiently detect the hemorrhage focus in the patients with CAAH and the diagnostic value is better than routine MRI.