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.     

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.     

烧结工艺对液相合成（SrPb）TiO3基陶瓷热敏特性影响的研究

本文在液相合成（ＳｒＰｂ）ＴｉＯ３粉的基础上，研究了升温速率、保温时间、烧结温度及降温方式对（ＳｒＰｂ）ＴｉＯ３基陶瓷的Ｖ型ＰＴＣ热敏特性的影响。实验表明：升温速率对其热敏特性影响不大，保温时间和烧结温度的作用是一致的，具有一最佳范围。而降浊方式对材料的热敏特性影响较大。综合各因素的影响，本配方中，样品的烧结制度定为：以２００℃／ｈ的速率升温，在１２５０℃保温１ｈ，炉冷至９５０℃，保温０．５ｈ，再     

LD钢超塑性压缩变形参数与金相组织

通过对ＬＤ（７Ｃｒ７Ｍｏ３Ｖ２Ｓｉ）模具钢起塑性压缩变形后的金相组织观察研究，分析了变形参数、原始组织对变形后金相组织的影响。     

铁电存储场效应晶体管I-V特性的物理机制模拟

文章讨论的模型主要描述了铁电存储场效应晶体管（FEMFET）的I-V特性。从理论结果可反映出几何尺寸效应和材料参数对晶体管电特性的影响。传统的阈值电压的概念巳不再适用，由于铁电层反偏偶极子的开关作用，自发极化的增加对存储器的工作状态产生很小的影响。该模型可用于设计和工艺参数的优化，并由直观原型的方法得到了验证。     

Breakdown characteristics of oil and paper insulation for a very fast transient voltage

This paper describes the dielectric breakdown characteristics of oil and oil‐impregnated paper for very fast transient (VFT) voltages. Blumlein circuits generate VFT voltages of 60 and 300 ns in a pulse width that simulates disconnecting switching surges in gas‐insulated switch gears. We measured the breakdown voltages of needle‐to‐plane, plane‐to‐plane oil gaps and several pieces of paper between plane electrodes for VFT and lightning impulse voltages. The measured data were formulated in V‐t characteristics and Weibull probability distributions. The inclination n of V‐t characteristics of insulating paper is 150, which is less than n = 13.7 of the plane‐to‐plane oil gap in the VFT time range. The shape parameters of Weibull distribution obtained in this study show that the scattering of breakdown voltages of paper is much less than that of oil. © 2002 Wiley Periodicals, Inc. Electr Eng Jpn, 141(4): 16–24, 2002; Published online in Wiley InterScience ( www.interscience.wiley.com ). DOI 10.1002/eej.10043