Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common hereditary cerebral small vessel disease.NOTCH3 missense mutation causes its coded cysteine occurring odd change and then affects the conformation and function of protein of NOTCH3.The abnormal NOTCH3 protein has vascular smooth muscle toxicity and finally deposits in the cerebral small blood vessels and causes the disease.Usually,CADASIL can be suspected by its typical clinical manifestations and neuroimaging findings.Its diagnosis needs genetic testing or skin biopsy to find the outer granular osmiophilic deposits of small vascular smooth muscle cells or immunohistochemical NOTCH3-ECD staining positive.For nearly two decades,the studies on genetics,pathogenesis,clinical manifestations,and diagnostic techniques of CADASIL have made great progress,however,many important questions have not been fully clarified and have new discoveries,such as the NOTCH3 gene mutation pattern and loci,and the relationship between gene phenotype and clinical phenotype,optimization of diagnosis process,depth study of pathogenic mechanism,exploration of new discoveries,new therapeutic targets and concepts.This article reviews the genetic characteristics,pathogenesis,and clinical diagnosis and treatment technology of CADASIL.

ObjectiveTo investigate long-term outcome after severe traumatic brain injury (TBI) with or without posttraumatic seizure (PTS). Methods225 inpatients of severe TBI were analyzed retrospectively, with parts of them followed up for 2～5 years after injury.Results31 cases (13.8%) were diagnosed as PTS. All the PTS patients and 66 TBI patients without PTS were followed up. There were lower scores of quality of life index (QLI) in PTS patients (5.23±2.25) than those without PTS (7.05±2.28).ConclusionThere was a worse long-term outcome in TBI patients with PTS.

ObjectiveTo analyze the prevalence of urinary incontinence in stroke patients in the convalescent stage, and the characteristics of different type of incontinence. Methods176 stroke patients in the convalescent stage were classified as urinary incontinence and continence, the former were classified as with and without awareness of the need to void. Their features were reviewed. ResultsThe incidence of urinary incontinence was 26.1%. No differences were observed for sex, types, and locations of the focus. There was statistic difference of the single/multiple lesions between the incontinence and the continence （P<0.05）. There was statistic difference in parietal lobe lesions and cognitive dysfunction among the patients with and without awareness of the need to void （P<0.05）. ConclusionThe urinary incontinence in stroke patients in the convalescent stage tend to occur in the patients with multiple lesions, and those without awareness of the need to void is associated with parietal lobe and the cognitive dysfunction.

Objective To design simulated pulmonary air embolism demonstration model,so as to solve the problem of pathological anatomy of pulmonary air embolism in experiment teaching. Methods According to the principle of the disturbance of local blood circulation and air embolism, we designed a pulmonary air embolism model. We took 223 school nursing students as the object of this study,and randomly divided them into 2 groups:animal experiment teaching group and model control group,then we compared the teaching effect between the two groups. Result The test scores of students in the animal experiment teaching group were higher than control group (P<0.05) . Conclusion The use of simulated pulmonary air embolism demonstration model teaching can improve the students’experimental test scores,and can be repeatedly used,stimulate students' study interest,reduce the cost of teaching,and improve the teaching quality.

Objective To study the novel methods of VMAT planning based dose volume histogram ( DVH) optimization, evaluated the dosimetry and planning efficiency in VMAT planning for Esophageal Carcinoma. Methods Twelve Esophageal carcinoma patients were enrolled in this study. The conventional VMAT planning as the reference, using multi?criterion optimization DVH ( MCO?DVH ) and overlapping volume histogram prediction DVH ( OVH?DVH ) two different methods to get ideal objectives function for informing VMAT plans, Then evaluate the dosimetric, planning efficiency for all new VMAT plans. The difference between the paired t?test groups. Results The two VMAT plans based DVH objective function can meet the clinical needs. Compared with the conventional VMAT plan, Conformity index ( CI ) and Homogeneity index ( 0. 77 vs. 0. 72, P=0. 017 and 0. 10 vs. 0. 12, P=0. 047 ) is better in DVH informed plans;lung V5 and spinal cord V50 are better in MCO?DVH informed plan (54. 66 vs.60. 23,P=0. 013 and 0. 98 vs.0. 49,P=0. 037).Furthermore,the DVH informed plans had higher planning efficiency (8. 2 vs. 19. 5,P=0. 023) . Conclusions DVH Objective informed VMAT Planning can achieve clinical needs with much uniform dose to target,lower OAR dose and higher planning efficiency.

Objective:To compare the efficacy and costs of pegylated recombinant human granulocyte colony stimulating factor (PEG-rhG-CSF) and granulocyte colony stimulating factor (G-CSF) for hematopoietic stem cell mobilization and hematopoietic recovery after transplantation in patients with relapsed or refractory malignant lymphoma. Methods:From July 2014 to October 2016, 15 patients with malignant lymphoma using peripheral blood stem cell mobilization (PBSCM) for autologous peripheral stem cell transplantation (APBSCT) were treated in our institution and enrolled in the PEG-rhG-CSF group (experimental group). We analyzed data from other 15 patients with malignant lymphoma mobilized with G-CSF who were treated in our institution from January 2013 to August 2015 (control group). Results:Patients in both groups were successfully mobilized. The median amounts of CD34+cells collected in the experimental and control groups were 16.2×106/kg and 8.9×106/kg, respectively (P=0.414), and the median amount of mononuclear cell (MNC) was 12.4×108/kg and 9.9× 108/kg, respectively (P=0.519). In the experimental and control groups, the mean durations of mobilization were 10.66±1.45 and 9.33±1.83 days (P=0.234), the mean durations of neutropenia during mobilization were 4.20±2.17 and 3.80±2.04 days (P=0.608), the mean durations of absolute neutrophil count recovery after APBSCT were 10.14±1.29 and 10.93±2.69 days (P=0.327), and the mean durations of platelet recovery were 10.36±2.27 and 12.27±3.38 days (P=0.121). Mobilization and hematopoietic recovery after APBSCT were not significantly different between the two groups. The cost was lower in the experimental group than that in the control group (RMB 3,960 yuan versus RMB 11,479.3±2,401.3 yuan). Conclusion:High-dose chemotherapy combined with PEG-rhG-CSF is a promising, effective, and low-cost mobilization regimen for patients with relapsed or refractory malignant lymphoma.

Objective To summarize the characteristics of medical service patterns at Grenada station of Mission Harmony-2015 so as to provide reference for future overseas services in similar island countries .Methods After detailed analysis of actual conditions of the countries visited and efficient communication with host country health organizations , we made an appropriate adjustment of the medical service pattern , which involved multi-site screening of general patients , followed by intensive checkups and treatment aboard the hospital ship .Results The procedure of diagnosis and treatment was streamlined and the efficacy of medical services was significantly enhanced to meet the maximum medical demand of local residents.Grenada became a station with the largest number of patients who received top-quality fee-free healthcare during Mission Harmony-2015 .Conclusion Adjustment of medical service patterns according to the actual conditions could deliver a desired effect during overseas missions .

OBJECTIVETo study the effect of platelet-derived growth factor-BB (PDGFBBB) on rat corpus cavernosum smooth muscle (CCSM) cell proliferation, migration and phenotypic modulation and explore the underlying mechanisms.

METHODSWistar rat CCSM cells were obtained through a modified tissue culture method and identified by immunofluorescence assay. The effect of PDGFBB on the proliferation of CCSM cells was investigated using a CCK-8 kit and the optimum PDGFBB concentration for cell treatment was determined. CCSM cells were treated with vehicle or PDGF-BB at the optimum concentration, and the cell migration was examined using scratch assay; the mRNA expression of the transcription factor myocardin and the contractile phenotype markers αSMA and SMMHC in CCSM cells were determined by qRT-PCR at 24 h and 48 h. The protein expression of myocardin in CCSM cells incubated with PDGFBB for 0, 24 and 48 h was examined by Western blotting.

RESULTIn CCSM cell culture, 96.5%and 96% of the cells were positive for αSMA and smoothelin, respectively. PDGFBB at different concentrations markedly promoted the proliferation of CCSM cells; the optimum PDGFBB concentration for enhancing cell proliferation was 12.5 ng/mL, which induced the migration of CCSM cells and significantly reduced the mRNA expressions of myocardin, αSMA and SMMHC (P<0.01). Exposure to PDGFBB decreased the protein expression of myocardin as the exposure time extended (within 48 h).

CONCLUSIONCCSM cells of a high purity can be obtained by the modified tissue culture method. PDGFBB can promote the proliferation and migration of CCSM cells and cause a phenotypic conversion from the contractile to the synthetic type possibly by down-regulating myocardin.

Actins ; metabolism ; Animals ; Cell Movement ; drug effects ; Cell Proliferation ; drug effects ; Cells, Cultured ; Down-Regulation ; Male ; Myocytes, Smooth Muscle ; cytology ; drug effects ; Myosin Heavy Chains ; metabolism ; Nuclear Proteins ; metabolism ; Penis ; cytology ; Phenotype ; Proto-Oncogene Proteins c-sis ; pharmacology ; RNA, Messenger ; Rats ; Rats, Wistar ; Trans-Activators ; metabolism

Objective To investigate the effect of tail vein transplantation of human amniotic mesenchymal stem cells (hAMSCs) with different transforming growth factor (TGF)-β expressions on recovery of sciatic nerve function in peripheral nerve xenotransplantation mice.Methods The hAMSCs were isolated from amnion membranes by healthy mother donors and identified by fluorescence activated cell sorter.The up-regulated and down-regulated TGF-β lentiviral plasmids were constructed and transfected into the purified hAMSCs;hAMSCs with stable up-regulated or down-regulated TGF-β expression were constructed.The sciatic nerves of C57BL/6 mice were isolated and cut out,and sciatic nerves of SD rats were isolated and transplanted into the sciatic nerve defected C57BL/6 mice to construct peripheral nerve xeno-transplanted mice models;these mice models were divided into 4 groups (n=10)according to random number table:control group,hAMSCs treatment group,high-expressed TGF-βhAMSCs treatment group,and low-expressed TGF-β hAMSCs treatment group;one d before modeling,phosphate buffer saline (PBS) or hAMSCs re-suspension were drawn with a syringe and slowly pushed into the tail veins of mice for transplantation treatment;14 d after treatment,DigGait analysis system was used to evaluate the recovery of sciatic nerve function in each group of mice.Result Fourteen d after treatment,the sciatic nerve function index (SFI) of the high-expressed TGF-β hAMSCs treatment group (-25.820±0.286) was significantly higher than that of the low-expressed TGF-β hAMSCs treatment group (-33.413±0.920) and hAMSCs treatment group (-30.755±0.421,P＜0.05).Conclusion The tail vein transplantation of hAMSCs with TGF-β high expression can effectively improve the sciatic nerve function in peripheral nerve xenotransplantation mice,which may be a new breakthrough in the treatment of peripheral nerve defects.

Objective:To evaluate the role of miR-20a-5p in M1 microglia aggravating oxygen-glucose deprivation and restoration (OGD/R)-induced injury to neurons and the relationship with mitofusin2 (MFN2).Methods:The well-growing BV2 microglia (M0 type) were polarized into M1 phenotype by lipopolysaccharide (100 ng/ml) and IFN-γ (20 ng/ml) and identified by quantitative real-time polymerase chain reaction and immunofluorescence.The well-growing N2a cells were divided into 6 groups ( n=6 each) by the random number table method: control group (group C), OGD/R group, M0 microglia co-culture group (group M0), M1 microglia co-culture group (group M1), miR-20a-5p inhibitor transfection group (group I) and negative control group (group NC). The cells were routinely cultured in group C, and the cells were subjected to OGD for 3 h followed by restoration of oxygen-glucose supply to develop the model of OGD/R injury in group OGD/R.The cells were subjected to OGD for 3 h and were co-cultured with M0 microglia for 24 h during restoration of oxygen-glucose supply in group M0.The cells were subjected to OGD for 3 h and were co-cultured with M1 microglia for 24 h during restoration of oxygen-glucose supply in group M1.In group I and group NC, cells were transfected with miR-20a-5p inhibitor and negative control miRNA into M1 microglia, respectively, and N2a cells were subjected to OGD for 3 h and co-cultured with M1 microglia for 24 h during restoration of oxygen-glucose supply.The cell viability was determined by cell counting kit-8 assay, amount of lactate dehydrogenase (LDH) released was determined, the expression of miR-20a-5p and MFN2 mRNA was detected by quantitative real-time polymerase chain reaction, and MFN2 expression was detected by Western blot. Results:Compared with group C, the cell viability was significantly decreased, the amount of LDH released was increased, and the expression of MFN2 protein and mRNA was down-regulated in the other five groups, miR-20a-5p expression was significantly up-regulated in OGD/R, M0 and M1 groups, and miR-20a-5p expression was significantly down-regulated in group I ( P<0.05). There were no significant differences in the cell viability, amount of LDH released, and expression of miR-20a-5p, MFN2 protein and mRNA between group OGD/R and group M0 ( P>0.05). Compared with group OGD/R and group M0, the cell viability was significantly decreased, the amount of LDH released was increased, and the expression of MFN2 protein and mRNA was down-regulated, and miR-20a-5p expression was up-regulated in group M1 ( P<0.05). Compared with group M1, the cell viability was significantly increased, the amount of LDH released was decreased, the expression of MFN2 protein and mRNA was up-regulated, and miR-20a-5p expression was down-regulated in group I ( P<0.05). Conclusions:The mechanism by which M1 microglia aggravates OGD/R-induced damage to N2a cells may be related to the up-regulation of miR-20a-5p expression in M1 microglia and the inhibition of MFN2 expression in N2a cells.