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.     

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.     

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.     

可调偏迈步式胶带自移机尾的研究

随着掘进工作面自动化程度的提高,掘进巷道速度也在日益加快,而现有的顺槽皮带机机尾移动缓慢且移动时工作量大,需要两三人配合操作,存在一定的安全隐患。针对以上问题,研发可调偏迈步式胶带自移机尾,具有与悬挂二运皮带转载机搭接行程长,可以迈步式前进,自动调偏等功能,同时,可以降低工人的劳动强度,加快巷道掘进速度。     

皮带秤秤架的响应特性曲线分析

介绍了皮带秤秤架响应特性曲线分析方法，对四种结构形式的秤架进行了响应特性曲线分析和比较，得出的结论是：悬浮式秤架的计量特性最好。     

Methods of Verifying Continuous Automatic Belt Weighers: State and Prospects

The basic methods of verifying continuous automatic belt weighers are described. A comparative analysis of these methods on the basis of experimental studies is made and ways of implementing the results in industry are recommended.     

HE-AAC V2编码原理及其应用

在保证优秀的音频质量下,提高音频编码的压缩效率是数字音频技术一直追求的目标,简单介绍目前最先进的音频编码HE-AAC V2的原理及应用,着重介绍其中用到的两种新技术频带复制(SBR)技术和参数立体声(PS)技术.     

浅谈延迟焦化装置露天栈桥的设计

根据延迟焦化装置露天栈桥的工程设计实践,围绕一炉两塔(一个加热炉两个焦炭塔)的焦化装置,分析了露天栈桥设计中的要点及难点,对其在平面布置、基础安排和吊车梁选型等设计方面做一些介绍和论述。