Background Choroidal neovascularization (CNV) is a serious complication of many fundus diseases.A variety of factors are associated with CNV.Research showed that recombinant human endostatin ( rhendostatin ) can arrest the vascular endothelial growth factor (VEGF) pathway and inhibit the proliferation of endothelial cells and angiogenesis.Objective This study was to observe the inhibition of rh-endostatin on experimental CNV.Methods The CNV animal models were created by Argon laser with the wavelength 532 nm to irradiate the inferior retina away optical disc 1-2 DD for 25 spots in 32 eyes of 16 chinchilla rabbits.The laser parameters were as follows:power 800 mW,spot diameter 75 μm and time shutter 50 ms.The models were then divided into model control group and rh-endostatin group.Rh-endostatin was intravitreously injected via scleral incision in 16 eyes of 8 model rabbits at 1 week after photocoagulation.Fundus fluorescein angiography (FFA) and optical coherence topography(OCT) were performed at 1,2,4 weeks after photocoagulation respectively.The eyeballs were enucleated and the retinal sections were prepared for the histopathologieal examination,and the contents of VEGF and pigment epithelial derived factor(PEDF) in rabbit vitreous,and blood serum were detected by ELISA at 2,4 weeks after photocoagulation.Results Retinal edema and exudes were seen in 1 week and scarring in 4 weeks after photocoagulation.In rh-endostatin injection group,the hyperfluorescence masses were seen in the background phase and early arterial phase in 42％ (84/200) of spots in the first week.The fluorescence leakage was decreased in the rh-endostatin injection group compared with control group in the second week and ceased at the third week on the FFA after injection.Variety forms of hyperreflective zones were found below the retinal pigment epithelium on the seventh day after photocoagulation.But the partial vessel occlusion and fibroplasias were identified in the rh-endostatin injection group in the third week by the OCT.The histopathological examination showed that the morphological abnormality was mild in the rh-endostatin injection group in comparison with model group.The serum PEDF concentration was significantly elevated but the VEGF/PEDF values in vitreous and serum were declined in rhendostatin injection group compared with model group (P ＜ 0.0 1 ).Conclusions Argon laser photocoagulation could induce the experimental CNV in chinchilla rabbit.Intravitreous injection of rh-endostar can effectively inhibit laser-induced CNV in rabbit.

Objective To observe the apoptosis of Th1 and Th2 cells in C57BL/6 mice chronically infected with Schistosoma japonicum.Methods The apoptotic Th1 and Th2 cells in spleen and lymph node from C57BL/6 mice infected with Schistosoma japonicum for 13 weeks were examined by three-color and indirect flow cytometery with staining surface molecule and intracellular cytokines.Results Compared with the normal mice,the proportion of apoptotic Th1 and Th2 cells of 13-week post-infection was significantly high,and the apoptotic Th1 cells increased more than apoptotic Th2 cells in the infected C57BL/6 mice,and the Th1 cells were more susceptible to apoptosis than Th2 cells.Conclusions Unequal susceptibility to apoptosis in Th1 and Th2 cells may be one of the reasons leading to Th2 polarization on mice chronically infected with Schistosoma japonicum,which provides the new proof of Th polarization.

Objective:To evaluate the safety and feasibility of the in-situ needle fenestration combined with the in vitro physician modified fenestration technique to reconstruct supra-aortic branches during thoracic endovascular aortic repair (TEVAR) for aortic arch lesions requiring landing at Z0 and Z1.Methods:From Nov 2017 to Dec 2019, eighteen patients who underwent both the in-situ needle fenestration and the in vitro physician modified fenestration techniques to extend the proximal landing zone to Z0 and Z1 during TEVAR were included in our study.Results:Sixteen patients underwent in vitro physician modified fenestration ,two patients underwent in vitro physician modified fenestration to reconstruct both the left common carotid artery and the innominate artery. All eighteen patients received in-situ needle fenestration to preserve the left subclavian artery. Supra aortic branches were preserved in all patients (38/38, 100%). There was no Type Ⅰ endoleak. Type Ⅱ endoleak was found in four paitnets (4/18). Type Ⅲ endoleak occurred in one patient (1/18). Type Ⅳ endoleak in four patients (4/18). Type Ⅲ endoleak needed open aortic arch repair 6 months later. The median follow-up time was 12 months. One (1/18) died in 12 months and the other patients were doing well.Conclusions:The joint application of the in-situ needle fenestration and the in vitro physician modified fenestration to reconstruct supra-aortic branches during TEVAR for aortic arch pathologies requiring landing at Z0 and Z1 was satisfactory.

Objective To analyze the growth phenotype and blood biochemical parameters of chromosome 1 substi-tution mouse strain(CSS1), and investigate their potential of QTL mapping .Methods Body weight, body length, tail length, organ weight of the CCS1 mice were measured at different days to create a growth curve while blood biochemical in -dexes were measured at about the 80th day.Results The CCS1 mice were different from C57BL/6 mice in several inde-xes.Compared with the C57BL/6 mice during different developmental stages , six strains including B6-Chr1KM mice were significantly different in body weight .There were five strains including B6-Chr1CM mice significantly different with C57BL/6 mice in body length, and all of the CSS1 mice were significantly different from C57BL/6 mice in tail length.Part of CCS1 mice were significantly different from C57BL/6 mice in the weight of liver, spleen, kidney and brain.The ALT of female B6-Chr1CM mice was significantly higher than that in the C 57BL/6 mice.The ALP of female B6-Chr1HZ mice was signifi-cantly higher than that in the male C57BL/6 and B6-Chr1KM mice, and was significantly lower than that in the C57BL/6 mice.The TB of male B6-Chr1CM, B6-Chr1SMX and B6-Chr1HZ mice was significantly higher than that of the C 57BL/6 mice.The TG of male B6-Chr1SMX mice and male B6-Chr1TW mice was significantly higher than that in the C 57BL/6 mice. Conclusions The phenotype of Chr1 CSS mice is quite different from commonly used inbred strain C 57BL/6 mice.CCS1 mice show great potential in QTL mapping for their characteristic growth phenotype and blood biochemical indexes .

AIMTo study the expression of leptin receptor (OB-R) and neuronal damage following focal ischemia/reperfusion in rats.

METHODS20 adult male Wistar rats were divided into four groups randomly: sham-operated 24 h,72 h control group and ischemic/reperfusion 24 h, 72 h experiment group. Focal ischemia/reperfusion model was made with MCAO. Immunohistochemistry and immunoelectron microscope were used to observe the expression of OB-R of the cortex and neuronal damage.

RESULTSThe positive cells of OB-R were found in pyramidal cells of the parietal cortex, choroid plexus and blood vessel endothelium. Compared with sham-operated group, significant reduction of OB-R positive cells in the pyramidal cells was observed in the ischemia/reperfusion rats 24 hours after cerebral ischemia (P < 0.05). The positive cells of OB-R of sham-operated 72 h group reduced further (P < 0.01). Histochemistry and electron microscope showed neuronal damage in the core area of cerebral ischemia in the late period was more obvious than in the early period.

CONCLUSIONThe early and delayed ischemia/reperfusion neuronal damage were accompanied with reduction of OB-R expression. Thus, it is worth to study the effect of OB-R in cerebral ischemia.

Animals ; Brain Ischemia ; genetics ; metabolism ; pathology ; Cerebral Cortex ; metabolism ; pathology ; Disease Models, Animal ; Gene Expression ; Male ; Neurons ; pathology ; Rats ; Rats, Wistar ; Receptors, Leptin ; metabolism ; Reperfusion Injury ; metabolism ; pathology

Objective To clarify the clinical features,therapeutic strategy and prognosis of lipid storage myopathy (LSM).Methods The clinical data and therapeutic effects of 42 LSM patients were summarized retrospectively.All patients were followed up to evaluate their prognosis.Results Data of short-term therapeutic results of all the 42 patients were available.Thirty-three cases were placed in low- doses prednisone and 9 cases in riboflavin.All patients showed marked and quick improvement of symptoms within one month.Among thirty-two patients followed up for more than one year,26 cases had a full recovery and 6 remained to have intolerance to heavy exercise.Thirteen patients had relapses of muscle weakness in various degrees and most of which were induced by exertion,exposure to coldness and upper respiratory tract infection.In 5 patients the symptoms were recurred for more than one time.Among 13 cases with relapses, 7 had family history.Conclusions Our data suggest that LSM is a treatable disease and well responsive to low-doses prednisone.The disease tends to recur,especially in patients with family history.Glutaric aciduria type Ⅱ should be considered in LSM patients who are responsive well to riboflavin,indicating drug therapeutic strategy for LSM should be based on the etiology of the disease.

Objective To investigate the clinical,neuroimaging and myopathological features of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes(MELAS).Methods The clinical manifestations,neuroimaging and myopathological features of 31 patients with MELAS diagnosed in our Neuromuscular Center in the recent 7 years were retrospectively analyzed.A3243G point mutations were analyzed by RFLP method in 10 patients.Results ①Clinical features:There were 18 male patients and 13 female patients.The age of onset ranged from 3 to 43 years,averaging 21.9 years.The averaged duration was 4.9 years.Thirteen patients in this group had family history of maternal inheritance pattern.The main clinical manifestations included short stature(26 patients),recurrent headache and vomiting(24 patients), muscle weakness(22 patients),epileptic seizure(21 patients),cognitive decline(19 patients),visual disturbance(17 patients),sensorineural deafness(16 patients),ataxia(6 patients),psychiatric symptom (8 patients),external ophathalmoplegia(2 patients)and diabetes mellitus(9 patients).The serum CK level was slightly elevated in 6 patients,and the fasting blood lactic acid was increased in 15 of the 18 detected patients.②Neuroimaging features:The stroke-like lesions were mostly confined to cerebral cortex, including temporal lobe(24 patients),occipital lobe(21 patients),parietal lobe(12 patients)and frontal lobe(4 patients).Three patients had deep white matter involvement.Migrating stroke-like lesions were confirmed in 4 patients by repeated cranial CT/MRI examination.In addition,cerebral atrophy(17 patients)and bilateral basilar ganglion calcification(11 patients)were found.③Myopathological features: Scattered ragged red fibers(RRF)in various number were found in all the patients by MGT staining.Other founding included strongly SDH-reactive blood vessel(27 patients),COX enzyme deficiency(19 patients), and mild to moderate lipid storage in RRF(20 patients).④MtDNA analysis showed 9 patients with A3243G point mutation in all the detected 13 patients.Conclusion The clinical and neuroimaging features may offer important clue to the diagnosis of MELAS,but a definite diagnosis of MELAS relies on the myopathology and mtDNA mutation analysis.

Background About one third of congenital cataract is associated with inheriting factor.The inherited heterogeneity has been found in congenital cataract.To seek the pathogenic gene is essential for the gene therapy. Objective Present study was to map and identify the causal gene for autosomal dominant congenital cataract (ADCC) in a Chinese family. Methods The clinical features of all affected members in this family were examined.Blood samples were collected from eleven family members for genetic linkage analysis.Polymorphic microsatellite markers were selected from the regions which harbor all known loci linked with ADCC.Universal fluorescent-labeled M13 primer was used in linkage analysis.Direct genomic sequencing was used to evaluate the candidate gene for example CRYBB2 gene.This study followed Helsinki Declaration and was proved by Tianjin City Ethic Committee.Written informed consent was obtained from each SUbject before any medical procees. Results The maximum two-point LOD score of 1.20 was obtained for marker D22S315 (θ=0).The LOD score of 0.6 was obtained for marker D16S3068.No mutation in all exons of CRYBB2 gene was found in the family. Conclusion CRYBB2 gene associated with ADCC was excluded from the family.A genome-wide linkage screening should be conducted.Genotyping with microsatellite markers using Universal fluorescent-labeled M13 primer can decrease the cost and obtain the same result.

Objective To prepare a capillary electrophoresis sieving medium and apply it in GA118-16A genetic analyzer. Methods The white solid polyacrylamide (LPA) was prepared by polymerization and lyophilized. Through the swelling of the sol buffer, the sieving medium was obtained. The sieving medium was evaluated by 1) characterizing the parameters, including molecular weight, structure and viscosity, 2) applying in the GA118-16A genetic analyzer, including the spatial calibration, the spectral calibration and the STR analysis.. Results The prepared sieving medium Mw 1.8 x 105Da, Mn 1.2 x 105 Da, is of correct structure and high purity. The polydispersity was 1.5The spatial calibration and spectral calibration files can be established successfully in GA118-16A genetic analyzer, and the sieving medium can effectively separate the DNA fragments with 1bp difference. The STR profile is of sharp peaks, no impurity peaks, no tail, and no peak loss. Conclusion The sieving medium prepared by the method can be applied to domestic genetic analyzer such as GA118-16A.

·AIM:To evaluate and characterize the macular thickness and macular volume in patients of different stages of diabetic retinopathy with special - domain optical coherence tomography( SD-OCT) .·METHODS: Totally 40 patients ( 78 eyes ) with diabetic retinopathy were recruited in the study from January 2016 to January 2017 in our hospital. According to the international clinical classification of diabetic retinopathy, 20 cases (40 eyes) were categorized as non-proliferative diabetic retinopathy ( NPDR ) group and 20 cases proliferative diabetic retinopathy (PDR) group (38 eyes). All subjects were examined and analyzed with Early Treatment Diabetic Retinopathy Study ( ETDRS ) subfields, which were embedded in HS ( Haag-Streit ) with diameter of 1, 3 and 6mm. The changes of retinal thickness and volume of the macular center were measured.·RESULTS: The thickness of macular foveolar in NPDR group and PDR group were 252. 57 ± 31. 36μm, 362. 47 ± 20. 81μm. The retinal thickness of inner superior subfield (ISM) and inner nasal subfield(INM) were the thickest;that of inner inferior subfield ( IIM ) was next to ISM and INM, and that of inner temporal subfield was the thinnest. Of the outer subfields, the retinal thickness of outer superior subfield ( OSM ) was the thickest;that of outer nasal subfield( ONM) was next to OSM, and that of outer temporal subfield(OTM)and outer inferior subfield ( OIM ) was the thinnest. The value of macular central concave thickness and retinal thickness in each quadrant of the NPDR group were less than those of the PDR group, the difference was statistically significant ( P <0. 05). The volume (V) of macular center in NPDR group and PDR group were 0. 20±0. 02mm3, 0. 28±0. 16mm3, the upper and nasal sides of the middle part of the partition were the largest, the inferior and the temporal side were the smallest. The nasal side of the outer loop was the largest, the upper was the second, the temporal side and the inferior were the smallest. The volume of macular central fovea and the retinal volume in each quadrant of the NPDR group were smaller than those of the PDR group, the difference was statistically significant (P<0. 05).·CONCLUSION: The changes of retinal thickness and volume in macular central fovea were related with the progression of diabetic retinopathy. Using OCT to analyze the macular thickness and macular volume in different stages of diabetic retinopathy, helps physicians to understand the morphological changes of macular region and its surrounding macular degeneration with the severity of diabetic retinopathy, and provide a basis for better analysis of the changes of the structure of macular in different severity diabetic retinopathy.