Clinical and genetic analysis of two Chinese families with benign familial neonatal convulsions

Benignfamilialneonatalconvulsions(BFNC)is arareautosomaldominantinheritedepilepsysyn dromecharacterizedbyunprovokedpartialorgeneral izedseizures.Theseizuresusuallyoccurfromthesec onddayofbirthtothesixthmonthandremitsponta neouslyafterseveralweekstomonths.Mostindivid ualsareseizure freebytheageofsixmonths.The serumchemistryandneuroradiologicalexaminations,interictalelectroencephalogram(EEG),andpsy chomotordevelopmentareusuallynormal.However,10%to15%ofpatientshavetheriskofseizurere currencela…     

荷电水雾饱和带电量的研究

本文利用液体表面性质及球体表面电荷的规律，从理论上讨论了荷电水雾的饱和带电量、雾滴极限荷质比及提高荷质比的措施．     

程控电网参数综合分析仪

<正> 电力系统各电参数的检测和分析,一般要用不同的电工仪表分别测量得到,当需要同时测量各参数时,显得十分麻烦。另一方面随着电力负荷的日益加重、大功率设备的不断投入使用,电力谐波干扰更加严重,急需对谐波分量进行分析和测试,为了满足电力部门和计量部门检测、分析和管理的需要,我们设计并研制了本仪器。一、主要功能和技术指标电压范围:0～1000V,误差±0.2％电流范围:0～500A,误差±0.2％功率(单、三相):0～500KW,误差±0.5％本仪器原理先进,智能化程度高,量程范围大,功能齐全。又加上具有自动故障检测,量程自动切换,配有标准接口和打印机接口等功能。特别是具有不断电测试功能和谐波分析功能,一问世就深受用户的欢迎,因此,在我国将会有广阔的应用     

Re-emerging superconductivity at 48 kelvin in iron chalcogenides

Pressure has an essential role in the production and control of superconductivity in iron-based superconductors. Substitution of a large cation by a smaller rare-earth ion to simulate the pressure effect has raised the superconducting transition temperature T(c) to a record high of 55?K in these materials. In the same way as T(c) exhibits a bell-shaped curve of dependence on chemical doping, pressure-tuned T(c) typically drops monotonically after passing the optimal pressure. Here we report that in the superconducting iron chalcogenides, a second superconducting phase suddenly re-emerges above 11.5?GPa, after the T(c) drops from the first maximum of 32?K at 1?GPa. The T(c) of the re-emerging superconducting phase is considerably higher than the first maximum, reaching 48.0-48.7?K for Tl(0.6)Rb(0.4)Fe(1.67)Se(2), K(0.8)Fe(1.7)Se(2) and K(0.8)Fe(1.78)Se(2).     

Blasting technologies of vibration damping in subway tunnel passing under upper buildings closely

Reducing the blasting vibration is important for blasting excavation in subway tunnel construction. Taking the 3rd bid section of Line 3 of Qingdao subway project as an example, the distance between tunnel vault and ground is 5 - 8 m. In order to insure the safety of the upper buildings, technologies of parallel cut with large diameter empty hole, one-time initiation and delay by parts, and multiple shallow holes were adop- ted in the project. The results showed that the maximum value of vertical vibration was limited in the criterion allowance, and the upper buildings were not damaged. Besides, problems were solved that the number of nonel detonator was difficult to meet the requirements of excavating a large cross-section tunnel by blasting, multiple cross-section could＇ t be initiated simultaneously, and construction efficiency was low, which ensure the con- struction safety and schedule.     

Induction of human β-defensin-2 in airway epithelial cells by Streptococcus pneumoniae： involvement of IP3-dependent intracellular calcium release and NF-κB

Human β-definsin-2 （hBD-2） is mainly induced by bacterial factors and pro-inflammation mediators in epithelial cells. As the major cause of community-acquired pneumonia, whether Streptococcus pneumoniae （S. pneu-moniae） stimulation induces hBD-2 expression in airway epithelial cells is elusive. In this study, we found that S. pneumoniae stimulation induced hBD-2 expression in a time-and concentration-dependent manner in primary human airway epithelial cells. To further reveal the mechanism of S. pneumoniae inducing hBD-2, we found that S. pneumoniae stimulation activated NF-κB signaling pathway. Specific NF-κB inhibitor, PDTC, could reverse the induction of hBD-2 by S. pneumoniae. We also found that cellular inner Ca^2＋ signaling is involved in the S. pneumoniae-induced hBD-2. Taken together, our find-ings indicated that S. pneumoniae can stimulate the expression of hBD-2 in airway epithelial cells and NF-κB and inositol triphosphate-dependent intracellular calcium release is involved in this induction.     

Effect of heating rate on the electrochemical performance of MnO X @CNF nanocomposites as supercapacitor electrodes

Carbon nanofibers （CNFs） and MnOjc@CNF nanocomposites （MCNFs） are fabricated by electrospin- ning and investigated as free-standing electrodes for sup- ercapacitor. This work presents the effect of heating rate during carbonization on the electrochemical behavior of the as-prepared MCNFs electrodes in 6 mol/L KOH elec-trolyte. Results show that the MCNFs electrodes carbon- ized by relatively slower heating rate exhibit higher specific capacitance. The electronic conductivity of the slow heated MCNFs electrodes is better than that of the fast heated electrodes due to the better crystallinity of the MnOx nanoparticles and the graphitic carbon layers forming on the surface of the Mn-loaded CNFs. These MCNFs electrodes demonstrate elevated rate capability and improved cycling performance without adding any polymer binder or electronic conductor.