Objective To study the relationship between the levels of serum oxide and antioxide in patients with Wilson disease (WD) and its visceral injury.Methods We determined the serum levels of MDA,GSH PX,GSH and VitE in 29 patients with WD and 21 normal controls by the method of reversephase high performance liquid chromatography (RP HPLC) and the chemo colorimetric method.Results The serum levels of MDA in all patients with WD were significantly higher than those of controls( P 0 05 ).Conclusion There was metabolism disorder of free radicals (FRs) in the serum of patients with WD.As compared with normal controls the serum levels of oxides increased and the levels of antioxides decreased in patients with WD,it might be one of important factors for the patients with visce ral injury.

Objective The selective deficit of recognition of disgust expression in Huntington’s disease (HD) and HD gene carrier strongly suggested the possibility that basal ganglia is involved in disgust processing. To further tested this hypothesis, we test the perception of emotional expressions in patients with symptomatic Wilson’s disease (WD), another basal ganglia disease resulted from abnormal copper metabo lism. Methods We developed a six basic emotional morphed face continuum (30 morphed emotional faces across happy surprise fear sad disgust anger happy). Participants were required to label each of these morphs in 5 trails to measure the ability of different emotion processing. The performances of 32 cases of WD were compared with 20 age and education matched normal controls. Results On the group level, although significantly impaired in fear, disgust and anger (correct identification scores: 12.8?3.6;9.1?4.7;14.9?4.3, P

Objective To study the hypothalamic-pituitary-ovary axis function in female patients with hepatolenticular degeneration (HLD). Method By RIA test the levels of serum pituitary and sex hormones were observed. Results The level of fallicle-stimulating hormone (FSH) was significantly lower than that in control group ( P

Somatostatin,a cyclopeptide hormone,is generally distributed in vivo.Some studies have indicated that somatostatin can reduce proinflammatory cytokine in hepatic stellate cells and extracellular matrix,and inhibit their proliferation and contraction,promote their apoptosis.It can also regulate the function of Kupffer cells,influence the homing of hepatic stem cells.Somatostain has therapeutic effect on liver fibrosis,especially on the liver fibrosis caused by schistosoma mansoni.

The aim of our study was to observe changes of biliary trace elements and hepatolienal ultrasonography in patients with HL D treated synthetically by metal-binding a- gents DMSA and DMPS.Using Aloka SSD2 5 6 ultrasonography and WFX-1 E2 atomic ab- sorption spectrophotometer,we analysed and asseyed the sonograms and biliary trace ele- ments of30 patients with differentultrasonographic typesbefore and six weeksafter DMSA and DMPS treatment.Biliary B juice were obtained by duodenal drainage.The results showed that the biliary copper content of patients with glinting echogenic dots type,rock stratum type and tree-like type increaed markedly after therapy(P

Objective To study the regional cerebral blood flow (rCBF) and correlated factors in patients with hepatolenticular degeneration (HLD).Methods The rCBF of lentiform nucleus,thalamus and other sites in 14 patients with HLD of cerebral type (cerebral type group) and 10 patients with HLD of non-cerebral type (non-cerebral type group) were determined by magnetic resonance-perfusion imaging technology,meanwhile 13 healthy volunteers were selected as control group,and calculated the relative regional cerebral blood flow (rrCBF) for avoiding perfusion time lag.The correlation between the clinical symptom scores,the content of urinary copper,duration and rrCBF in HLD patients were evaluated.Results The rrCBF of cerebral type group in the left and right frontal lobe,temporal lobe,lentiform nucleus,caudate nucleus,thalamus,midbrain,pons and the left hippocampus,cerebellar cortex,dentate nucleus were lower than those of control group (1.91 ±0.35 vs.2.44 ±0.64,1.80 ±0.30 vs.2.37 ±0.65,1.37 ±0.35 vs.2.14 ±0.91,1.58 ±0.52 vs.2.39 ±0.99,1.61 ±0.38 vs.2.59 ±0.74,1.52 ±0.64 vs.2.63 ±0.73,1.88 ±0.32 vs.2.61 ±0.67,1.70 ±0.40 vs.2.35 ±0.50,1.48 ±0.13 vs.2.01 ±0.59,1.46 ±0.38 vs.2.38 ±0.99,1.47 ±0.55 vs.2.02 ±0.72,1.27 ±0.48 vs.1.91 ±0.51,1.24 ±0.38 vs.1.47 ±0.29,1.25 ±0.39 vs.1.53 ±0.37,1.74 ±0.27 vs.2.40 ±0.89,1.79 ±0.50 vs.2.22 ±0.66,2.15 ±0.41 vs.2.64 ± 0.61),and there were significant differences (P ＜ 0.05 or ＜ 0.01).There were no significant differences in the rrCBF of the parietal and occipital lobe,etc between cerebral type group and control group (P ＞ 0.05).The rrCBF of cerebral type group in the left and right lentiform nucleus were lower than those of non-cerebral type group (1.61 ± 0.38 vs.1.94 ± 0.58,1.52 ± 0.64 vs.1.99 ± 0.59),and there were significant differences (P ＜ 0.05).The clinical symptom scores were positively correlated with the rrCBF of the left and right lentiform nucleus in 24 patients with HLD (r =-0.792 and-0.764,P ＜ 0.01),the content of urinary copper and duration had no correlation with the rrCBF of the left and right lentiform nucleus(P ＞ 0.05).Conclusions The rCBF of cerebral type and non-cerebral type HLD is significantly reduced,cerebral type patients have lower rCBF than non-cerebral type patients.The rCBF is correlated with the clinical symptom scores.

Objective To investigate the changes of cardiac structure and function in patients with hepatolenticular degeneration (HLD).Methods From March 2010 to February 2011,90 HLD patients who did not receive formal treatment (observation group) and 30 healthy people (control group) were collected and analyzed with color Doppler echocardiography in the Institute of Neurology,Affiliated Hospital of Anhui University of Traditional Chinese Medicine.Based on the clinical manifestations,cases were divided into hepatic type group (n =31),brain type group (n =42) and brain-visceral type group (n =17).According to the Child-Pugh classification,patients were divided into Child A group (n =71),Child B group (n =10) and Child C group (n =9).Results (1) The left atrial diameter ((28.00 ± 3.11),(29.62 ± 3.44) mm) and left ventricular diameter ((45.69 ± 3.75),(47.10-± 4.73) mm) of HLD patients in brain-visceral type group and hepatic type group were larger than that of the control group ((24.86 ± 2.63),(41.93 ±3.56) mm;t =3.143,4.761,P=0.018,0.000;t=3.764,5.167,P=0.018,0.000).The left atrial diameter and left ventricular diameter of hepatic type group were significantly larger than the brain type group (26.06 ± 3.68,43.34 ± 3.88;t =3.557,P =0.000;t =3.751,P =0.001).The value of E/A in the hepatic type group (1.57 ± 0.37) was significantly lower than the control group (1.93 ± 0.20;t =-0.352,P =0.006).(2) The left atrial diameter ((31.29 ± 1.70),(34.67 ± 1.97) mm) and left ventricular diameter ((48.29 ± 2.81),(53.67 ± 2.67) mm) of Child B and C groups were significantly larger than the control group ((24.86 ± 2.63),(41.93 ± 3.56) rm;t =6.429,9.810,P =0.000,0.000;t =6.357,10.738,P =0.000,0.000),and the Child A group ((26.42 ± 3.05),(43.89 ± 3.76) rm;t=4.871,8.252,P=0.000,0.000;t =4.399,8.780,P=0.003,0.000).The value of E/A of Child B and C groups (1.58 ± 0.32,1.26 ± 0.39) was lower than that of the control group (t =-0.347,0.662,P=0.020,0.000);At the same time,the value of E/A of Child C group was significantly lower than that of Child A group (1.80 ± 0.33;t =-0.530,P =0.000).Conclusions The HLD patients may have cardiac structural and functional changes,mainly manifested as left atrial and left ventricular enlargement and cardiac diastolic dysfunction,whereas a serious impact has not yet been found in systolic function.And the changes of cardiac structure and function were related to the degree of liver cirrhosis in patients with HLD.

Objective: To explore the feasibility of using 3D transesophageal echocardiography (3D-TEE) data to generate 3D patient-specific model of mitral valve (MV) using 3D printing. Methods: A retrospective study was performed in 30 patients, including 10 cases with normal MV, 10 cases with MV stenosis and 10 cases with MV prolapse. 3D-TEE volumetric data of 30 patients were acquired and postprocessed, and then soft MV models were made through injecting silicone into the molds. Measurements of MV annulus antero-posterior diameters, anterolaterior and posteromedial diameters, perimeter, area and MV opening width obtained from 3D-TEE images and from 3D models were compared, and the absolute differences of these values were calculated. Results: 3D-TEE volumetric data were successfully postprocessed, and then 3D MV models were made in all patients. The differences of MV parameters measured from 3D models were not statistically significant with those from 3D-TEE images (all P>0.05). Only less absolute differences of MV parameters were found between 3D models and 3D-TEE images. Conclusion: 3D printing of MV models based on 3D-TEE is feasible with high conserved fidelity.

Objective: To analyze the relationship between tumor deposits(TD) and clinicopathological features of locally advanced gastric cancer(LAGC) patients. Methods: The clinicopathological characteristics of 300 LAGC patients undergoing radical gastrectomy in Renmin Hospital of Wuhan University from Jan 2014 to Jul 2017 were collected. The relationship between clinicopathological features and prognosis of LAGC patients and TD status in LAGC patients were analyzed retrospectively. Results: TD were positive in 65(21.7%) patients. Univariate analysis showed that the depth of invasion, lymph node metastasis, pTNM stage, tumor size, vascular invasion, preoperative tumor markers were correlated with the status of TD(all P<0.05). Multivariate Logistic regression analysis showed that lymph node metastasis, pTNM stage, vascular invasion and depth of invasion were independent risk factors for positive TD (allP<0.05). Univariate analysis on prognosis of LAGC patients showed lesion location, degree of differentiation, lymph node metastasis, pTNM stage, tumor size, positive TD, number of TD and vascular invasion are associated with prognosis of LAGC (all P<0.05). Multivariate COX regression analysis showed that the number of TD >3 was an independent risk factor for the prognosis of LAGC patients(HR=2.242, 95% CI: 1.484-3.386, P=0.000). The median survival time of TD-positive LAGC was less than TD-negative group(χ²=91.077, P<0.001). The median survival time of TD number>3 group was significantly shorter than that in TD number<3 group and TD-negative group(χ²=192.969, P<0.001). Conclusions TD are closely related to tumor stage in LAGC patients. The status of TD is an independent risk factor for the prognosis of LAGC. Multiple TDs indicate poor prognosis of LAGC patients.

Objective: To observe the regulatory effects of RhoA/ROCK pathway on the apoptosis of cardiac myocyte induced by anoxia and its mechanism. Methods: The model of cardiac myocyte anoxia was established. The beat pulsations and apoptosis rates after 1 h, 3 h, 6 h, 9 h and 12 h of anoxia were recorded and the expressions of RhoA, ROCK1/2, p-PI3K, p-AKT and caspae-3 were detected, too. The apoptosis and the expressions of related proteins were detected after RNAi of RhoA and the inhibition of ROCK by Y-27632. Results: The beat pulsations after 1 h, 3 h, 6 h, 9 h and 12 h decreased gradually but the apoptosis rates increased gradually, and the expressions of RhoA, ROCK1/2, p-PI3K, p-AKT and caspase-3 were increasing along with the increasing duration of anoxia. The apoptotic rates after 1 h, 3 h, 6 h, 9 h and 12 h of anoxia were (4.36[U+4F9D]0.98)%, (8.36[U+4F9D]2.12)%, (15.32[U+4F9D]3.62)%, (18.68[U+4F9D]4.83)% and (24.56[U+4F9D]6.22)%, respectively and decreased more significantly than control group in different time points of anoxia (. P<0.05), and the expressions of RhoA, ROCK1/2, p-PI3K, p-AKT and caspase-3 decreased significantly (. P<0.05). The apoptosis rate and the expressions of RhoA, ROCK1/2, p-PI3K, p-AKT and caspase-3 decreased significantly (. P<0.05) after the inhibition of ROCK by Y-27632 (. P<0.05). Conclusions: RhoA/ROCK pathway plays a critical role in the regulation of the apoptosis of cardiac myocyte induced by anoxia, which may be accompanied by regulating the activity of PI3K/AKT/Caspase-3 pathway.