New Insights into the Pathogenesis of Systemic Mastocytosis

Mastocytosis is a type of myeloid neoplasm characterized by the clonal, neoplastic proliferation of morphologically and immunophenotypically abnormal mast cells that infiltrate one or more organ systems. Systemic mastocytosis (SM) is a more aggressive variant of mastocytosis with extracutaneous involvement, which might be associated with multi-organ dysfunction or failure and shortened survival. Over 80% of patients with SM carry the KIT D816V mutation. However, the KIT D816V mutation serves as a weak oncogene and appears to be a late event in the pathogenesis of mastocytosis. The management of SM is highly individualized and was largely palliative for patients without a targeted form of therapy in past decades. Targeted therapy with midostaurin, a multiple kinase inhibitor that inhibits KIT, has demonstrated efficacy in patients with advanced SM. This led to the recent approval of midostaurin by the United States Food and Drug Administration and European Medicines Agency. However, the overall survival of patients treated with midostaurin remains unsatisfactory. The identification of genetic and epigenetic alterations and understanding their interactions and the molecular mechanisms involved in mastocytosis is necessary to develop rationally targeted therapeutic strategies. This review briefly summarizes recent developments in the understanding of SM pathogenesis and potential treatment strategies for patients with SM.     

寒冷地区某既有办公建筑围护结构优化改造研究

既有公共建筑能耗中,围护结构节能潜力巨大。以兰州某既有办公建筑为例,采用DeST-C软件模拟围护结构不同材料厚度、窗型,得出系列负荷,通过对数据分析、比较,得出如下结论:外墙外保温适宜材料为50mm的挤塑板,最大热负荷节能率为45.66%;屋面适宜保温材料为65mm的挤塑板,最大热负荷节能率为4.25%;外窗各个朝向全部更换玻璃,推荐选用内张膜中空玻璃(双膜),最大热负荷节能率为7.36%;单独更换南向外窗材料,推荐选用真空镀膜复合中空玻璃,最大热负荷节能率为1.78%。     

Accounting for the uncertainties in the estimation of average shear wave velocity using V S– N correlations

Site-specific seismic hazard analysis is crucial for designing earthquake resistance structures, particularly in seismically active regions. Shear wave velocity ( V _S) is a key parameter in such analysis, although the economy and other factors restrict its direct field measurement in many cases. Various V _S–SPT– N correlations are routinely incorporated in seismic hazard analysis to estimate the value of V _S. However, many uncertainties question the reliability of these estimated V _S values. This paper comes up with a statistical approach to take care of such uncertainties involved in V _S calculations. The measured SPT– N values from all the critical boreholes were converted into statistical parameters and passed through various correlations to estimate V _S at different depths. The effect of different soil layers in the boreholes on the Vs estimation was also taken into account. Further, the average shear wave velocity of the top 30 m soil cover ( V _S30) is estimated after accounting for various epistemic and aleatoric uncertainties. The scattering nature of the V _S values estimated using different V _S– N correlations was reduced significantly with the application of the methodology. Study results further clearly demonstrated the potential of the approach to eliminate various uncertainties involved in the estimation of V _S30 using general and soil-specific correlations.     

一种基于FPGA的可变速率帧结构设计与实现

在简述V.35接口的基础上针对V.35接口速率可变的应用需求提出了一种速率可变的帧结构,该帧结构可支持N×64kb/s(3≤N≤32)速率,从而在V.35接口上实现了多种速率的低速业务传输.     

两种3D打印用雾化Ti-6Al-4V粉末的对比研究

本研究分别利用水冷铜坩埚真空感应熔炼气雾化(VIGA-CC)和等离子旋转电极(PREP)两种技术制备出球形Ti-6Al-4V合金粉末,作者利用SEM、同步辐射CT扫描-三维重建和氩气含量测试等分析手段对不同粒径的Ti-6Al-4V合金粉末的孔洞缺陷和氩气含量、硬度值进行了表征。实验结果表明, VIGA-CC粉末粒度分布宽,细粉收得率较多,粉末粒度分布在40~180 μm之间, PREP粉末的粒度分布较窄,主要集中在110~180 μm之间；金属粉末内部的孔隙率、气体含量和孔尺寸随着粉末粒度的增大而增大,且同一粒径范围内VIGA-CC粉末的气孔概率多于PREP粉末；随着粉末粒径减小,粉末截面组织逐渐细化,其硬度值逐渐升高,整体上VIGA-CC粉末硬度值高于PREP粉末。     

Formation and wear mechanism of nickel titanium intermetallics during heat treatment of nickel coating on Ti-6Al-4V substrate

In the present work, inter-diffusion of nickel and titanium and formation of Ni-Ti intermetallic compounds on Ti-6Al-4V substrate have been studied. Initially, nickel was electrodeposited on the alloy using a modified Watts bath solution at a current density of 2 A dm^?2 for 1?h. The coated specimens were then heat treated for different durations at 750, 800 and 850 °C under argon atmosphere. The effects of temperature and time on the characteristics, hardness and wear resistance of intermetallic phases were investigated. The results showed that a multilayer structure was formed after heat treatment, an outer layer of residual nickel, an area of intermetallic layers with different compositions followed by a solid solution of Ni-Ti. It was also observed that an increase in time or temperature at first led to the formation of thicker intermetallic layers; however, after passing a critical point, the intermetallic layers seem to dissolve into the substrate. Furthermore, the wear rates of the diffusion treated samples were four times lower compared to Ti-6Al-4V alloy when sliding against AISI 52100 hardened steel.     

Linear friction welding of Ti6Al4V: experiments and modelling

Linear friction welding of the Ti6Al4V alloy is studied. A new definition of the energy input rate is proposed, based on an integration over time of the in-plane force and velocity; a strong correlation with the upset rate is then found. The effective friction coefficient is estimated to be 0·5±0·1 for varying frequencies and amplitudes, with only a weak dependence on the processing conditions displayed. A model is proposed that accounts for both the conditioning and equilibrium stages of the process, which is shown to be in good agreement with the experimental data. The model is used to study the mechanism by which the flash is formed. A criterion is proposed by which the rippled nature of its morphology can be predicted.     

基于步冷试验的2.25Cr-1Mo-0.25V钢母材及焊缝回火脆化研究

对原始态、步冷态、脱脆态和脱脆步冷态2.25Cr-1Mo-0.25V钢母材及焊缝冲击试验结果进行分析,得到了母材和焊缝在不同状态下的韧脆转变温度vTr54.2和FATT以及脆化度ΔvTr54.2和ΔFATT。试验结果表明,步冷试验之后,母材发生较低程度脆化或脱脆现象,但是脆化度或脱脆度较低,表明母材具有良好的抗回火脆化性能;经脱脆试验后,母材和焊缝都发生较高程度的脱脆,表明材料的脆化主要是由于回火脆化引起的,脱脆试验使得材料的韧脆转变温度降低;脱脆步冷试验后,焊缝发生较高程度的脆化,焊缝对脱脆步冷试验的敏感性较高,脱脆步冷试验有效促进了焊缝的脆化。在相同脆化条件下,母材的脆化敏感性低于焊缝,焊缝更易发生脆化。