Objective To observe the expression of HER-2 and TOPO-Ⅱα in ovarian epithelial cancer,analyze the correlation between their expression and provide theoretical basis for clinical diagnosis,prognosis and treatment. Methods Expression levels of HER-2 and TOPO- Ⅱα in 10 normal ovarian tissues,20 benign tumors and 58 cases of ovarian epithelial cancers were detected by immunohistochemical method, and their correlations with pathological features were analyzed. Results The positive expression rate of HER-2 in normal ovarian and benign tumor tissues were significantly lower than ovarian epithelial cancers respectively ( 10. 0% , 15.0% VS 46. 6% ;P ＜ 0. 05 ). The positive expression rate of TOPO- Ⅱα in ovarian epithelial cancers was significantly higher than normal and benign epithelial ovarian tumor tissue (53.4% vs 10. 0%, and 15.0%,Ps ＜ 0. 05 ), but we did not find significant difference in the comparison between normal and benign epithelial ovarian tumor tissue ( Ps ＞ 0. 05 ). The expression of HER-2 and TOPO- Ⅱα were significantly correlated with clinical stages, histological differentiation of tumor cells (Ps ＜ 0. 05 ) ,but there were no correlations between the age or histological type. In ovarian cancer tissues, a positive correlation between the expression of HER-2 and TOPO- Ⅱα was observed ( r = 0. 324, P ＜ 0. 05 ) . Conclusion The overexpression of HER-2 and TOPO- Ⅱαplay an important role in ovarian carcinogenesis and development. The expression of HER-2 is positively correlated with TOPO- Ⅱα in ovarian epithelial cancers. Coexpression of the two moleculars may be involved in the development and progression of ovarian epithelial cancer, which should be further studied.

Objective:To evaluate the endothelial functions and autoregulation capacity of cerebral blood flow in patients with Fabry disease .Methods:Brachial artery vasodilation was assessed in 8 pa-tients with Fabry disease and 14 healthy controls by means of flow-mediated dilation ( FMD) and Nitro-glycerin-mediated dilation ( NMD) .Cerebrovascular reactivity was calculated in terms of breath-holding index ( BHI) and vascular motor reactivity ( VMR) by TCD-CO2 test in 4 patients and 14 healthy con-trols.Results:Compared with the controls , brachial artery vasodilation experiment showed no difference (the patients:FMD 15.94%±5.03% and NMD 23.92%±7.23%, the controls: FMD 14.57%± 5 .84% and NMD 22 .64%±6 .96%) , there was no relationship between FMD or NMD and the age , course of disease , MSSI or enzyme activity .In respect of cerebrovascular autoregulation capacity , there was no difference in anterior circulation , while cerebrovascular reactivity tended to be impaired in posteri-or circulation .Conclusion:Endothelial function showed no decline in patients with Fabry disease , but cerebrovascular autoregulation capacity tended to be impaired in posterior circulation .

Objective To analyze membrane attack complex (MAC) expression in different types of idiopathic inflammtory myopathy (IIM).Methods We enrolled 57 cases of dermatomyositis (DM) , 37 cases of polymyositis (PM) ,9 cases of sporadic inclusion body myositis (sIBM) and 15 cases of autoimmune necrotizing myopathy with anti-signal recognition particle antibodies (SRP-ANM) in Department of Neurology at Peking University First Hospital from 2011 to 2014, and used x2 test or Fisher exact test to analyze MAC expression in muscle fibers and endomysial capillaries respectively.Results The total MAC expression in DM, PM, sIBM and SRP-ANM was 75.4％ (43/57), 86.5％ (32/37), 4/9 and 13/15 respectively.The MAC expression in muscle fibers was 50.9％ (29/57), 81.1％ (30/37) , 3/9 and 13/15 respectively.The MAC expression in endomysial capillaries was 49.1％ (28/57) , 24.3％ (9/37) , 1/9 and 6/15 respectively.The MAC expression in muscle fibers and endomysial capillaries of sIBM was less than other types of IIM.The MAC expression in muscle fibers of PM and SRP-ANM was higher than DM and sIBM, but there was no statistically significant difference between PM and SRP-ANM.The MAC expression in endomysial capillaries of DM and SRP-ANM was higher than PM and sIBM, while there was no statistically significant difference between DM and SRP-ANM (x2 =0.397, P =0.574).The MAC expression in four types of IIM had regional distribution, of which 11.6％ (5/43) of DM showed bundle distribution.Conclusion There are differences in the damage of MAC in various types of IIM, the damage of MAC in SRP-ANM indicated the pattern of both DM and PM.

Objective To report the clinical and peripheral neuropathological findings in two patients with autosomal recessive Charcot-Marie-Tooth disease 2K(AR-CMT2K).Methods Case one was a nine year-old girl.She had distal weakness of lower limbs for six years, with calf atrophy and contracture of Achilles tendon for three years.Case two was an eight year-old boy.He had distal weakness of lower limbs with contracture of Achilles tendon and calf muscle atrophy for three years, and proximal weakness of low limbs for two years.The motor nerve conduction velocities in median nerves were 48.1 m/s in case one and 47.6 m/s in case two.The compound motor action potential amplitude of median nerves decreased by 46% in case one and 69% in case two.Sural nerve biopsies and gene targeted next-generation sequencing were performed in both patients.Results Density of myelinated fibers was 8 407/mm2 in case one and 7 714/mm2 in case two.The ratio of myelinated fibers with diameter over 8 μm was 2.6% in case one and 0 in case two.Both patients had small regenerating cluster of myelinated fibers.Thin myelinated fibers appeared in case one.In case two, atypical onion bulb formations with focal folded myelin appeared, and electromicroscopy revealed mitochondrial aggregate in axons.Compound heterozygous mutations of ganglioside-induced differentiation associated protein 1 gene were detected in both patients, including c.767A>G(p.H256R) and c.466G>A (p.A156T) in case one and c.767A>G and 845G>A(p.R282H) in case two.Conclusions Contracture of Achilles tendon may appear in early childhood of AR-CMT2K patients.The main pathological changes in sural nerve are loss of large myelinated fibers, mitochondrial aggregate in axons and myelin abnormalities.

ObjectiveTo investigate the pathologic characteristics of the sural nerve in amyotrophic lateral sclerosis. MethodsClinical, electrophysiologic, laboratory data and sural nerve biopsy of 11 patients were reviewed. The clinical and laboratory data were compatible with the diagnosis of ALS. The sural nerve was removed and immediately fixed in 10% formalin and phosphate-buffered 2.5% glutaraldehyde and processed according to the procedure used in our laboratory for light and ultrastructural examination.Results4 groups were distinguished based on pathologic changes: normal; with mild axon degeneration and demyelination; with mild loss of the myelinated nerve, axon degeneration and demyelination; with severe loss of the myelinated nerve, axon degeneration and demyelination.ConclusionPredominantly axonal neuropathies are common and occur early in ALS. Axon degeneration of the nerve fibers is predominant, and demyelination also can be performed in patients with ALS.

Objective To summarize the clinical features of Chinese patients with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes ( MELAS).Methods A total of 190 patients with MELAS who presented to Peking University First Hospital between 1997 and 2015 were recruited.Among 190 patients, 175 were identified carrying mitochondrial DNA mutations, and the remaining 15 patients were diagnosed by muscle biopsy.The clinical features, including predisposing factors of stroke-like episodes, the onset symptoms and frequencies of various manifestations were analyzed and reported.Results In our cohort of MELAS patients, the male-to-female ratio was 1.44∶1.The median age of onset was 14 years ( from 7 months to 45 years).The peak onset ages were 8-12 years.The median onset age of the first stroke-like episode was 16 years ( from 1 to 53 years ).There were 66 ( 46.15%) patients who had predisposing factors before the onset, and fatigue and upper respiratory tract infection were the most common predisposing factors of stroke-like episodes in these patients ( 37.88%, 25/66 and 34.85%, 23/66, respectively).Other predisposing factors included emotional agitation, drinking alcohol, trauma, withdrawal of antiepileptic drugs, being frightened, satiation and hunger.Stroke-like episodes appeared in 70.53%(134/190) patients as an onset symptom and developed in all patients with disease progression.The neurological manifestations included seizure ( 89.42%, 169/189 ) , mental retardation or dementia (82.87%, 150/181), headache (74.30%, 133/179), hemianopia or cortical blindness (67.72%, 107/158), exercise intolerance (50.87%, 88/173), hemiplegia or hemianesthesia (47.44%, 74/156), sensorineural deafness (46.20%, 85/184), aphasia (39.47%, 60/152), behaviour disorder (17.71%, 31/175) and ophthalmoplegia ( 9.60%, 17/177 ).The manifestations of extra-nervous systems included hirsutism (67.57%, 100/148), vomiting (65.58%, 101/154), fever (62.07%, 90/145), short stature (45.32%, 63/139), diarrhea or constipation (43.48%, 70/161), low body mass index (26.62%, 37/139), diabetes mellitus (20.79%, 37/178) and kidney disease (3.16%, 6/190).Conclusions The majority of the patients in this study have the disease onset during childhood.There are more male MELAS patients than females.Most common clinical manifestations are seizure, mental retardation or dementia, headache, cortical blindness, hirsutism, vomiting and fever in this patient group.

Objective:To investigate the protective effects of levosimendan combined with lyophilized recombinant human brain natriuretic peptide (rhBNP) on myocardium in patients with acute myocardial infarction complicated by heart failure.Methods:140 patients with acute myocardial infarction complicated by heart failure who received treatment in Changxing People's Hospital from June 2018 to June 2020 were included in this study. They were randomly assigned to receive either routine treatment (control group, n = 70) or routine treatment, levosimendan combined with rhBNP (study group, n = 70). Serum levels of creatine kinase (CK)-MB, cardiac troponin I (cTnI), lactate dehydrogenase (LDH), high-sensitivity C-reactive protein (hs-CRP), which were associated with myocardial injury, were measured in each group. In addition, the changes in cardiac ultrasound indexes left ventricular end diastolic diameter (LVEDD), left ventricular end systolic diameter (LVESD) and left ventricular ejection fraction (LVEF) were observed. Clinical effects on heart failure were evaluated. Adverse drug reactions were monitored during the treatment. Results:After treatment, CK-MB, cTnI, LDH and hs-CRP levels in the study group were (56.73 ± 12.15) U/L, (0.41 ± 0.19) μg/L, (126.83 ± 15.26) U/L and (1.59 ± 0.27) mg/L, respectively, which were significantly lower than those in the control group [(78.52 ± 14.07) U/L, (0.68 ± 0.21) μg/L, (187.25 ± 23.04) U/L, (2.84 ± 0.41) mg/L, t = 5.569-12.418, all P < 0.05]. LVEDD and LVESD in the study group were significantly lower than those in the control group, while LVEF in the study group was significantly higher than that in the control group ( t = 4.435-6.426, all P < 0.05). Total effective rate in the study group was significantly higher than that in the control group [88.57% (62/70) vs. 72.86% (51/70), χ2 = 5.552, P < 0.05]. There was no significant difference in total incidence of adverse drug reactions between study and control groups [11.43% (8/70) vs. 8.57% (6/70), χ2 = 0.317, P > 0.05]. Conclusion:Levosimendan combined with rhBNP can effectively alleviate myocardial injury in patients with acute myocardial infarction complicated by heart failure, improve myocardial function, is highly safe, and thereby deserves clinical application.

Objective To analyze abnormal characteristics of mitral valve (MV) caused by rheumatic and degenerative mitral valvedisease in virtue of the real-time three-dimensional transesophageal echocardiography(RT3DTEE) and quantitative analysis software.Methods Sixty patients underwent RT3DTEE study:21 patients with rheumatic valvular disease(RHD) studied intraoperatively(9 severe mitral stenosis,6 severe mitral regurgitation,6 severe mitral stenosis couple with regurgitation ) and 20 patients with normal MV who were used as controlsubjects(NS).TomTec-Arena 1.0-4D MV-Assessment 2.3 software was used to measure parameters of annular dimensions and geometry,[eafletsurface area and so on.Results Compared with NS,the diameter between anterior and posterior(AP) of RIID increased,however,the commissural width (CW)didn't change.Parameters of AP,CW,annulus circumference(AC) and annulus area(AA) of degenerative mitral valve disease(DMVD) increased obviously,the spherical index instead didn't change,the original "saddle" shapebecame flat.The impact on mitral valve leaflets is difference between RHD and DMVD.The anterior instead of posterior leaflet arena and length became larger of the group MS and MS&MI.However,the group of DMVD has increased leaflet area of anterior as well as posterior.Posterior leaflet angle is significantly increased in the group of MS&MI.The angle,between the DAP and plane of aortic valve annular (Angle AAo-AP),were magnified in both RHD and DMVD.From the automatic dynamic analysis,the parameters of annular displacement(max),annulus area fraction(2D)decreased considerably in RHD group.Conclusion RHD has relatively mild impact on annular of MV,but the lesions of mitral valve leaflet and chordae tendineae limit the movement of bicuspid valve an nular.The dilated and flattened annular of DMVD still has the "saddle" shape.

Objective:To investigate the safety and efficacy of J-valve transapical catheter aortic Valve replacement(TA-TAVI) in the early treatment of aortic stenosis with low coronary ostium.Methods:From January 2020 to April 2022, 20 patients with aortic stenosis with coronary opening height ≤10 mm who underwent TA-TAVI treatment in the Valve Surgery Center of Beijing Anzhen Hospital Affiliated to Capital Medical University were enrolled retrospectively. All patients underwent preoperative imaging screening, and 5 patients underwent preoperative coronary artery protection: The height of coronary artery opening was less than 5 mm in 3 cases. 2 cases had stent stenosis & LT after coronary artery stenting 50%, and the position of the coronary artery opening was less than 8mm. The postoperative complications, mortality and cardiac function prognosis of TA-TAVI were analyzed.Results:There were 5 males and 15 females. The average age of the whole group was(73.00±6.20) years. Transthoracic echocardiography showed that all patients had severe aortic valve stenosis, and 85% of the patients had NYHA grade Ⅲ-Ⅳ, and 80% of the patients had aortic valve stenosis with insufficiency. The mean height of left coronary artery opening was(9.07±3.70) mm. RCA(11.39±3.00) mm; The opening height of coronary artery was ≤5 mm in 3 cases(left 7.5%), 5-8 mm in 5 cases(12.5%), and 8-10mm in 16 cases(40.0%). Mean aortic sinus(valsalva) diameter: Left(30.06±5.90) mm; Right(28.50±5.68) mm; Non(29.96±6.15) mm. J-valve Valve was successfully implanted through apical catheter in all patients, most of whom were size 23. CPB was performed in 2 patients at the same time, permanent pacemaker was inserted in 2 patients, and moderate or above perivalvular leakage was found in 0 patients. All patients had TNI( P=0.12) and MYO( P=0.03) before and 24 hours after operation. None of the 5 patients underwent coronary artery stenting. None of the 5 patients died within 30 days after the operation. Ta-tavi is effective 30 days after operation, with low complication rate and good prognosis of cardiac function. Conclusion:J-valve transapical catheter aortic Valve replacement is safe and effective in the treatment of aortic stenosis with low coronary ostium.

Objective To explore the clinic results of our new designed transcatheter valve in valve for the mitral biopro-thesis deterioration.Methods Mitral bioprothesis deterioration patients with high risk for the routine bypass surgery got con-sent.After general anesthesia in the hybrid operation the left ventricle apical puncture was performed with guidance of 3D echo and X-ray.The retro-preset J Valve system was guided into the left atrium and the mitral bioprothesis with the beating heart . Results There were total 9 cases between Jan 2019 to Mar 2019 which had STS score above 6-8.The mitral bioprothesis in-cluded Hancock valve 3 cases, Perimount valve 3 cases, Epic valve 2 cases, Baxiter valve 1 case.The mitral bioprosthesis size included 27 for 6 cases and 25 for 3 cases.The successful implant rate was 100％, mortality rate 0, and all the patients recov-ered well without any main complications.The mean tran-valular pressure was only(8 ±2) mmHg (1 mmHg=0.133 kPa). Conclusion This innovative mitral valve in valve technique with retro-preset J Valve system got very good clinic results and worthy of deep research.