Sibling concordance for clinical features of Duchenne and Becker muscular dystrophies

Introduction: The correlation of markers of disease severity among brothers with Duchenne or Becker muscular dystrophy has implications for clinical guidance and clinical trials. Methods: Sibling pairs with Duchenne or Becker muscular dystrophy (n = 60) were compared for ages when they reached clinical milestones of disease progression, including ceased ambulation, scoliosis of ≥ 20°, and development of cardiomyopathy. Results: The median age at which younger brothers reached each milestone, compared with their older brothers ranged from 25 months younger for development of cardiomyopathy to 2 months older for ceased ambulation. For each additional month of ambulation by the older brother, the hazard of ceased ambulation by the younger brother decreased by 4%. Conclusions: The ages when siblings reach clinical milestones of disease vary widely between siblings. However, the time to ceased ambulation for older brothers predicts the time to ceased ambulation for their younger brothers. Muscle Nerve 49 : 814–821, 2014     

Inhibition of mTORC1 renders cardiac protection against lipopolysaccharide

Sepsis-induced cardiac dysfunction is a severe clinical problem. It is evident that rapamycin can protect heart from pathological injuries. However, there are no data demonstrating rapamycin reverse cardiac dysfunction induced by sepsis. In this study, Lipopolysaccharide (LPS) was administrated to mice and H9c2 cells. After treatment, we further determined cardiac function by echocardiography, ANP, BNP and inflammatory markers by qPCR and apoptosis by TUNEL staining. Moreover, mTORC1 signaling pathway and Akt activity were measured by Western blots. We found that rapamycin attenuated cardiac dysfunction, increase in ANP and BNP as well as apoptosis induced by LPS both in mice and in H9c2 cells. Unexpectedly, LPS did not significantly affect the mRNA levels of TNF-α and IL-6. Furthermore, rapamycin further reduced the decrease in mTORC1 signaling and Akt activity induced by LPS. In conclusion, rapamycin can protect heart from LPS induced damages by inhibition mTORC1 signaling and elevation of Akt activity.     

经验交流癫痫患者手机APP管理模式初探

为了促进河北省癫痫的诊疗管理水平,提高癫痫病患者生活质量.河北省抗癫痫协会通过手机APP,就疾病诊断、药物添加治疗及其疗效进行了多中心的临床数据分析研究.通过"核桃仁"健康管理平台对收集的临床数据进行总结分析,河北省的癫痫综合征的诊断率在半年内已从21％提升至39％,服药1个月内无发作率达80.4％,可看出医师对于癫痫...     

Controlling the adsorption of osteopontin for mediating cell behaviour by using self-assembled monolayers with varying surface chemistry

Osteopontin (OPN) is an important protein for mediating cell behaviour on biomaterials. However, the interactions between the chemical groups on the biomaterial surface and OPN still need to be further clarified, which has restricted the application of OPN in biomaterial functionalization. In the present study, we developed different self-assembled monolayers (SAMs) with specific chemical groups, including SAMs-OH, SAMs-OEG, SAMs-COOH, SAMs-NH₂, and SAMs-PO₃H₂, to study the behavior of OPN on these SAMs. The results showed that SAMs-NH₂ could strongly adsorb OPN, and the amount of protein was highest on this material. Meanwhile, the lowest amount of OPN was present on SAMs-OEG. Interestingly, the unit-mass trend of bound OPN monoclonal antibodies (mAbs) on the SAMs was opposite to the OPN adsorption trend: lowest on SAMs-NH₂ but highest on SAMs-OEG. In vitro cell assay results showed that mouse bone marrow mesenchymal stem cells (mBMSCs) on SAMs-COOH, SAMs-NH₂, and SAMs-PO₃H₂ with pre-adsorbed OPN showed promoted behaviour, in terms of spreading, viability, and the expression levels of α_v and β₃ genes, compared with the other two SAMs, demonstrating the higher bioactivity of the adsorbed OPN. We believe that our findings will have great potential for developing OPN-activated biomaterials.

Osteopontin (OPN) is an important protein for mediating cell behaviour on biomaterials.     

基于信息化的手术分级及非计划再手术管理智能实时监控研究

目的探索通过开发基于信息化的手术分级及非计划再手术管理智能实时监控系统,以提高手术管理的质量和效率。方法通过上海市嘉定区南翔医院开发的“南翔医院手术及非计划再手术智能监控系统1.0”,对,手术及非计划再手术进行实时监控。结果与传统的手术检查模式相比,基于信息化的手术分级及非计划再手术管理智能实时监控更为及时、有效,有助于提高检查效率和质量。结论基于信息化的手术分级及非计划再手术管理智能实时监控已具备现实可能性,可以将事后监控前伸,提高手术监管的效率和质量,对提高医疗质量、确保手术安全也能起到有效的作用。     

基于MRI动态图像观察吞咽时器官动度对头颈部肿瘤调强放疗靶区影响

目的 利用MRI技术连续采集头颈部肿瘤患者吞咽时图像,观察并测量软腭、舌、喉的运动规律及最大活动度。方法 随机选取2018年7月-10月在中国医学科学院肿瘤医院接受调强放疗的原发头颈部恶性肿瘤20例患者,其中男17例、女3例,中位年龄58.5岁(28~78岁)。20例患者中鼻咽癌7例,口腔癌3例,口咽癌5例,下咽癌3例,鼻腔鼻旁窦2例。根据AJCC第八版分期Ⅰ-Ⅱ期患者2例,Ⅲ期8例,Ⅳ期10例。结果 吞咽时软腭向上移动移动距离为(1.06±0.31) cm且服从正态分布,向后移动距离为(0.83±0.24) cm且近似正态分布。舌体向后移动距离为(0.77±0.22) cm,且服从正态分布。含压舌板行图像采集患者舌上移位移为0,无压舌板患者舌体中位上移距离为1.23 cm (0.59~1.41 cm)。喉向上移动距离为(1.14±0.22) cm且服从正态分布,向前移动的中位距离为0.4 cm (0.27~0.90 cm)。结论 吞咽运动有可能发生于头颈部肿瘤患者放疗过程中,并引起大体肿瘤体积(GTV)及周围正常组织移动;因此在制定放疗计划时应注意GTV至PGTV的个体化外放距离,以保证肿瘤处方剂量。     

基于全域医疗大数据的精准预警监管系统研究与实践

从总体框架、数据层架构、服务层模式等方面介绍基于全域医疗大数据的精准预警监管系统构建,阐述其应用实践,指出该系统应用能够提高数据效能、监管效率以及执法精准性,为相关研究提供参考。     

靶向沉默ATRX对辐射诱导宫颈癌HeLa细胞周期阻滞和凋亡的作用

目的:靶向沉默HeLa细胞中α-地中海贫血/精神发育迟滞综合征X染色相关蛋白(ATRX),探讨其对辐射诱导的细胞周期阻滞和细胞凋亡的作用.方法:利用RNA干扰(RNAi)技术建立稳定沉默ATRX的HeLa细胞模型,命名为shATRX1-HeLa,以shCon-HeLa为阴性对照.分别给予0、2和8 Gy X射线照射后,...     

共用净化空调对手术室空气环境的影响

对57例俯卧位脊柱手术患者进行术中眼部预防护理，包括加强术前宣教、术中选择合适的体位垫、正确摆放体位、使用反光镜观察病情、定时对面部支撑点的皮肤进行按摩。结果57例患者术中均未发生颜面部、眼部周围皮肤压疮及眼部并发症。术后观察随访1周，手术切口均Ⅰ期愈合。提出重视并做好俯卧位手术患者眼部防护可有效预防眼部并发症发生。