Objective Membranous nephropathy ( MN) is rarely complicated by crescents.This study was to observe the clinicopathologic characteristics of MN with crescents. Methods This retrospective study included 53 cases of biopsy-proven idiopathic MN with crescents in the absence of immunologic and clinical etiologic factors and another 100 MN patients without histological crescents as controls.The clinicopathologic features, treatment response, and out-comes were analyzed and compared between the two groups of pa-tients. Results Significantly higher percentages of hypertension and decreased eGFR were observed in the MN +crescents group than in the control (47.2%vs 19.0%, P<0.05;28.3%vs 40.%, P<0.05).Circulating autoantibodies against the M-type phospholipase A2 receptor (PLA2R) were found in 66.7%(30/45) of the patients.The glomeruli exhibited a median of 4.6%(1.8%-35.3%) involvement of crescents.Compared with the controls, the MN +crescents group showed remarkably higher rates of segmental glomer-ulosclerosis lesions (16.0%vs 49.1%, P<0.05), capillary loops necrosis (0.0%vs 11.3%, P<0.05), interstitial fibrosis/tubu-lar atrophy (IFTA) (54.0%vs 86.8%, P<0.05) and afferent arterial lesions (65.0%vs 92.5%, P<0.05).No significant differ-ences were found in the outcomes of the two groups of patients. Conclusion MN with crescents is rare, and secondary MN and cres-centic glomerulonephritis should be considered.Crescentic MN usually presents with hypertension and renal dysfunction clinically and high rates of severe segmental and global glomerulosclerosis, capillary loops necrosis, and IFTA histologically.The condition has a fa-vorable short-term prognosis.

Objective: To investigate the relationship between serum HGF levels and clinical severity of essential hypertension (EH). Methods: The serum HGF concentrations of 44 patients with EH were measur ed by ELISA. Results: The serum HGF levels in patients with EH w ere higher than that in control. Furthermore, the serum HGF levels of EH patient s with coronary atherosclerosis (CAS) were significantly higher than those of EH patients without CAS ［(920.8±250.0） pg/ml vs （747.9±132.1） pg/ml, P <0.01］ or control ［（643.8±98.2） pg/ml, P<0.01)］.The changes of HGF l evel were correlated with the clinical courses (r=0.63, P<0.01) and stag es (r=0.69, P<0.01) of hypertension. Conclusion: HGF may be considered as a new index for the severity of hypertension and an useful bio chemical parameter for estimating the development of atherosclerosis.

Objective To sum up the clinical information and outcomes of one-stage surgical repairs for interrupted aortic arch (IAA) associated with cardiac defects through median stemotomy in infant.Methods From August 2005 to January 2012,23 IAA patients,aged 18 days to 3 years [mean age (8.61 ± 11.81) months],body weight 3.3-13.0 kg [mean (6.61 ± 3.26) kg] were reviewed underwent one-stage repair.There were 12 male and 11 female.The anatomic subtype ineluded type A 20 (87.0％),type B 3 (13.0％),and no type C in the records.All cases included ventricular septal defect and patent ductus artefious,some also with artirical-pulmonary window,aterical septal defect,bicuspid or unicuspid aortic valve,and subvalvular aortic stenosis.Diagnosis was determined in of the patients and suspected in by echocardiography.Also,64 layers helico-CT was employed to make a definite diagnosis for 18 patients and cardiac catheterization was used for 4 patients.All patients with cardiac anomalies underwent one-stage repair through median sternotomy.The aortic continuity was reestablished by anastomosis between the descending aortic segment and aortic arch.Results In all 23 cases,21 were successful.There was 2 (8.69％) postoperative death:one was due to surgical hemorrhage and severe low cardiac output during perioperative stage and the other was 2-month old due to crisis of pulmonary hypertension.CPB time was ranged from 53-215 min [(129.76 ± 38.98) min],and aortic crossclamp time was 34-125 min[(74.47 ± 24.30) min].The length of stay in ICU postoperatively was 96h averagely.The postoperative complications included severe low cardiac output syndrome in 3 patients,hypoxemia in 13,pneumonia in 7,and supraventricular tachycardia in 12.21 patients were followed up from 2 months to 6 years and were in good condition without recoactation.Conclusion The outcomes of early and medium term for one-stage repair of IAA and associated cardiac anomalies through median stemotomy is excellent.Technique of extended anastomosis between the descending aortic segment and aortic arch may reduce the incidence of recoarctation.It is simplified the procedure and improved life quality of patients.

Objective To assess the results of emergency endovascular stent-grafting for patients with acute Stanford type B aortic dissection(type B AAD)within 24 hours of onset.Methods Between June 2007 and October 2008,30 patients with acute type B aortic dissection underwent emergency endOVascular stent-grafting within 24 hours of presentation.Under general anesthesia,stent-graft was deploved at the proper position of first tear entry through femoral artery under X-ray monitering.Follow-up by CT was performed 1 w,1 m ,3 m,6 m,1 y postoperatively to observe the efficacy and complications such as endoleak,migration and fracture of stent-graft. Result The technical success rate was 100%;13.4%(4 cases) endoleak rate was identified immediately after deployment.Follow up was made between 1 month to 19 months,averaging at(12±8)months,3.3%type-1 endoleak Was observed after 6 months;One patient died within 30 days possibly of dissection rupture;One patient died of acute liver failure during the follow-up. Conclusion Endovascular repair with stent-graft within 24 hours of presentation was effectivefor the treatment of acute type B aortic dissection.

The purpose of this study is to investigate the Sal I, Nru I and Mse I restriction fragment length polymorphisms (RFLPs) of factor IX gene in Chinese Han people. The frequencies of FIX-192 and FIX-793 for A and G, and FIX-698 for T and C were analyzed by polymerase chain reaction (PCR) in unrelated normal Chinese Han people. A sample of 214, 210 and 206 unrelated X chromosomes were analyzed for FIX-192 and FIX-793 and FIX-698, respectively. The results showed that the frequencies for FIX-192 were 0.878 for A and 0.122 for G, with a heterozygosity rate of 0.213, and the frequencies for FIX-793 were 0.552 for A and 0.448 for G, with a heterozygosity rate of 0.494, the frequencies for FIX-698 were 0.311 for T and 0.689 for C, with a heterozygosity rate of 0.429. It was concluded that the SalIand NruI and MseI RFLPs of FIX gene may be useful markers for carrier detection and prenatal diagnosis in Chinese families with hemophilia B patients.
China ; DNA ; genetics ; metabolism ; Deoxyribonucleases, Type II Site-Specific ; metabolism ; Factor IX ; genetics ; Female ; Gene Frequency ; Heterozygote ; Humans ; Male ; Polymorphism, Restriction Fragment Length

Objective To summarize the technique and clinical experience of hybrid procedure under cardiopulmonary bypass (CPB) in children with muscular ventricular septal defect (mVSD). Methods From January 2006 to June 2010, 45 cases of mVSD underwent hybrid procedure with CPB. mVSDs were closed with devices under direct vision in 45 cases. Of them, there were 20 males and 25 females. They ranged from 52 days to 12 years [mean (2.05 ±2.48) year] in age and from 3 to 30 kg [(11.93 ±7.70)kg] in body weight. Preoperatively, most of children were highly susceptible to respiratory tract infections. The hybrid approach was used in all patients with CPB under the guidance of transesophageal echocardiography (TEE). The diameter of mVSDs ranged from 2 to 7 mm under TEE. Of 45 cases, 40 patients had increased rates of pulmonary blood flow. 29 patients had left axis deviation and 12 cases had sinus arrhythmia on electrocardiography (ECG). 19 had other congenital heart lesions, including transposition of great arteries in 1 case, tetralogy of Fallot in 2, pulmonary artery stenosis in 3, patent ductus arteriosus in 6, atrial septal defects in 6) and aorta coactation in 1. The quantity of VSDs were from 1 to 7 (single, in 7; two, in 24 case; three, in 8 case; four, in 5 case and seven, in lease. 37 patients were combined with pulmonary hypertension in our cohort. 38 patients with another large VSD and 19 with other congenital heart lesions were required surgical repair at sometime. Results The hybrid procedures were undertaken in all 45 cases of this cohort. All cases were successful and no deaths occurred during operation. A total of 48 devices were implanted in 45 patients, including single devices in 42 cases (device size ranged from 4 to 10 mm) and two devices in 3 cases (device size ranged from 4 -7 mm). The average time on CPB was (58.28 ±20.70) min , while aortic crossclamp time was(34. 94 ± 14.75) min. In addition, the time on mechanical ventilation postoperatively ranged from 2 hours to 6 days. Compared to the older children, 20 infante aged less than 8 monhad a significant difference in cardiac function in the early postoperative period. One infant was given up treatment because of serious infection. Anather cases recovered with the use of supportive treatment, such as using vasoactive agents, digoxin, inhaling nitric oxide, diuresis, and so on. The enteric-coated aspirin was given at dose of 5 mg ? kg -1. day -1 for a period of 3 to 6 months as usual postoperatively. All patients attended follow-up at 1 week, 1 month, 3 months, 6 months, 1 year and 2 years post-procedure. No major complications were encountered during this period. All cases were no instance of migration of any of the devices, residual shunt, aortic regurgitation, atrioventricular valve dysfunction, Ⅲo atrial-ventricular conduction block, new arrhythmia, and so on. There are no death in long-term follow-up. Conclusion Hybrid procedure is safe and effective for the closure of congenital heart defects in children.

Objective: To investigate the relationship between serum HGF levels and clinical severity of essential hypertension (EH). Methods: The serum HGF concentrations of 44 patients with EH were measur ed by ELISA. Results: The serum HGF levels in patients with EH w ere higher than that in control. Furthermore, the serum HGF levels of EH patient s with coronary atherosclerosis (CAS) were significantly higher than those of EH patients without CAS ［(920.8±250.0） pg/ml vs （747.9±132.1） pg/ml, P <0.01］ or control ［（643.8±98.2） pg/ml, P<0.01)］.The changes of HGF l evel were correlated with the clinical courses (r=0.63, P<0.01) and stag es (r=0.69, P<0.01) of hypertension. Conclusion: HGF may be considered as a new index for the severity of hypertension and an useful bio chemical parameter for estimating the development of atherosclerosis.

Objective: To determine the eff ect of probucol on adhesion of human monocytic line THP-1 induced by oxidized low density lipoprotein (oxLDL). Methods: THP-1 cells were induced by oxLDL in vitro. The CD11b, CD54 expressions and adhesion to human umbilic al vein endothelial cells (HUVEC) were measured after treatment with probucol at different concentrations by flow cytometry and β-nitrophenyl N-acetyl-β-D -glucosminide test. Results: Probucol inhibited the adhesion of oxLDL-induced THP-1 cells to HUVEC and down regulated the expression of CD11b in a dose dependent manner (P<0.01), but there was no inhibition on exp ression of CD54. Conclusion: Probucol can inhibit adhesion and a ggregation of monocyte-macrophages to endothelium in circulation, and may have anti-inflammatory action.

Objective To identify abnormal cerebral glucose metabolism characteristics in patients with corticobasal degeneration (CBD) using 18 F-fluorodeoxyglucose (FDG) PET imaging. Methods From January 2014 to January 2017, resting-state brain 18 F-FDG PET imaging was performed in 10 CBD patients (5 males, 5 females; average age: (63.4±6.2) years) and 20 age-matched healthy subjects (8 males, 12 female; average age: (63.6±6.2) years). Voxel-based statistical parametric mapping (SPM) was used to analyze images to obtain the CBD-related brain metabolic characteristics. The regional cerebral metabolic rate of glucose (rCMRglc) was compared between 2 groups by two-sample t test. Results Compared with healthy controls, CBD group demonstrated asymmetrically decreased glucose metabolism mainly in the cere-bral hemisphere opposite to the more clinically affected body side, including the superior, middle and inferi-or frontal gyrus, the precentral gyrus, the superior and inferior parietal lobule, the angular gyrus, the supra-marginal gyrus, the precuneus, the middle occipital gyrus, the middle and inferior temporal gyrus, Heschl gyrus, the fusiform gyrus, the insula and the thalamus. And relatively increased glucose metabolism was present in ipsilateral precentral and postcentral gyrus, hippocampus, insula and putamen, bilateral cerebel-lum, paracentral lobules and pontine. The rCMRglc in those regions was significantly different between CBD patients and healthy controls (t values: 4.236-9.044, all P<0.01). Conclusion The asymmetric cerebral glucose metabolism features in CBD based on 18 F-FDG PET imaging contribute to the differential diagnosis between CBD patients and healthy subjects.

Objective To investigate the value of statistical parametric mapping (SPM) analysis of 18F-fluorodeoxyglucose (FDG) PET imaging in the differential diagnosis of Parkinsonism in single-case level.Methods SPM software was used to retrospectively analyze the 18F-FDG PET images of 160 patients (104males,56 females,age:30-82 years) who were suspected with Parkinsonism at baseline and were clinical confirmed by follow-up from April 2010 to December 2017.18F-FDG PET images of patients was compared with those of age-matched healthy controls in single-case level using two-sample t test in SPM software to obtain the imaging diagnosis.By comparing imaging diagnosis with the final clinical diagnosis,the diagnostic accuracy of SPM in the overall cohort as well as the early subcohort (duration of disease less than 2 years (56 males,22 females,age:50-82 years)) were calculated respectively.Results Among 160 patients with Parkinsonism,146(91.2％) had the same 18F-FDG PET diagnosis as their final clinical diagnosis.The diagnostic sensitivity for Parkinson's disease (PD),multiple system atrophy (MSA),progressive supranuclear palsy (PSP) and cortical basal ganglia degeneration (CBD) were 93.5％ (86/92),92.3％ (24/26),84.0％(21/25) and 15/17,respectively.The specificity were 95.6％(65/68),95.5％(128/134),96.3％ (130/135) and 100％(143/143),respectively.In the early subcohort,the analysis also achieved similar differential diagnosis effectiveness(92.3％).Conclmion The single-case 18F-FDG PET imaging SPM analysis can be helpful in the early differential diagnosis of Parkinsonism effectively.