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.     

Some Molecular and Cellular Stress Mechanisms Associated with Neurodegenerative Diseases and Atherosclerosis

Chronic stress is a combination of nonspecific adaptive reactions of the body to the influence of various adverse stress factors which disrupt its homeostasis, and it is also a corresponding state of the organism’s nervous system (or the body in general). We hypothesized that chronic stress may be one of the causes occurence of several molecular and cellular types of stress. We analyzed literary sources and considered most of these types of stress in our review article. We examined genes and mutations of nuclear and mitochondrial genomes and also molecular variants which lead to various types of stress. The end result of chronic stress can be metabolic disturbance in humans and animals, leading to accumulation of reactive oxygen species (ROS), oxidative stress, energy deficiency in cells (due to a decrease in ATP synthesis) and mitochondrial dysfunction. These changes can last for the lifetime and lead to severe pathologies, including neurodegenerative diseases and atherosclerosis. The analysis of literature allowed us to conclude that under the influence of chronic stress, metabolism in the human body can be disrupted, mutations of the mitochondrial and nuclear genome and dysfunction of cells and their compartments can occur. As a result of these processes, oxidative, genotoxic, and cellular stress can occur. Therefore, chronic stress can be one of the causes forthe occurrence and development of neurodegenerative diseases and atherosclerosis. In particular, chronic stress can play a large role in the occurrence and development of oxidative, genotoxic, and cellular types of stress.     

An Improved Immune Genetic Algorithm for Solving the Optimization Problems of Computer Communication Networks

1　IntroductionIndesigningacomputercommunicationnet work ,thenetworkaveragedelayisanimportantpa rameterinthenetworkperformance .Inthispaper,weonlyconsiderM /M/1networks,whichmeansthatthemessageprocessingtimeisaprobabilisticdensityfunctionwithnegativepower,thegroupar rivalandsendingisofPoissiondistributionwithasinglequeue .Supposethatthenetworktopologicalstructureandtheestimatesoftheexternaltrafficrequirementsaregiven ,howtoselecttheoptimalroutestobeusedbythecommunicatingnodesinthenetworksoast…     

南水北调西线工程可调水量分析

在受水区缺水形势严峻的情况下，引水工程可调水量的确定主要与三个因素有关：一是引水河流及引水坝址径流量；二是坝址上下游用水要求；三是引水水库工程规模。在考虑各种因素影响后，提出各引水坝址可调水量占坝比径流量的比例在70％左右比较合适，按现阶段推荐的两种方案，可调水量可达160亿－170亿m^3.     

一种抗NLOS的TDOA定位算法

本文提出了一种抗非视线传播(Non-Line-of-Sight)干扰的TDOA定位算法。该算法采用退火与遗传算法相结合的方式，在满足给定条件的空间内搜索源点坐标值。数值仿真表明，该算法具备抗NLOS干扰的能力，与传统TDOA算法相比，该算法无需知道各基站的非视线传播干扰分布，是一种实用有效的定位算法。     

27例乙型血友病患者Ⅸ因子基因突变研究

采用ＰＣＲ（聚合酶链反应）及ＧＡＷＴＳ（ＧｅｎｏｍｉｃＡｍｐｌｉｆｉｃａｔｉｏｎｗｉｔｈＴｒａｎｓｃｒｉｐｔｓＳｅｑｕｅｎｃｉｎｇ）技术，研究了江苏、湖北、山东、广东、福建、宁夏六省区２７例乙型血友病患者及其家系成员ＦⅨ（九因子）基因各２．２Ｋｂ　ＤＮＡ序列。在２ｌ例中发现２０种不同类型的突变。其中１２种是未曾报道的新突变。３８％的突变发生在ＣｐＧ二核苷致序列上，进一步证实了ＣｐＧ确系突变热点。同时检出６例女性为致病基因携带者。对开展基因产前诊断及优生优育等，具有重要意义。     

基于神经网络的降雨径流预报

本文将人工神经网络应用于二滩水电站以上支流鱼河的降雨径流预报，通过建模、计算、比较和分析可知，其非线性映射能力、自学习能力和灵活性，均能较好地反映降雨径流之间的非线性的、复杂的转换关系，结合流域水文特性并合理处理建模数据，值得进一步深入研究。     

川中丘陵区一种新型小流域径流输沙模型的建立与应用

径流输沙模型对定量评价水沙的输出响应具有重要的意义。本研究在已有研究成果的基础上，通过对四川盐亭县林山西沟小流域的径流、泥沙进行现场观测与分析，提出含沙量单位线的定义及其确定方法，建立了适应该小流域的径流输沙模型。并应用实测资料进行比较，表明模型的模拟结果可靠，模拟过程确定性系数达0．8以上，为川中丘陵区小流域径流输沙过程的预测预报提供了一个新方法。     

瀑布沟工程的水文分析计算

本文简要的介绍了瀑布沟工程水文分析计算主要依据站的选定和基本资料的插补延长,以及径流、洪水的分析计算方法.在可行性研究阶段水文成果(通过审查)和新设立的毛头码水文站所取得的数年水文资料的基础上,将初设阶段对可行性研究阶段的水文分析计算方法和成果进行验证和补充的情况重点予以介绍.     

黑河干流中游地区耗水量变化的历史分析

黑河流域水资源极其匮乏，加之地区上的配置不均衡和中游地区水土资源的过度开发，导致黑河下游出现了严重的生态环境问题，已影响到我国北方广大地区生态环境质量，受到国家的高度重视和全社会的广泛关注。黑河水资源开发利用主要集中在中游地区，该区是全国重要的商品粮、蔬菜和经济作物生产基地之一，耗水量占流域耗水量的85％以上，是黑河的主要耗水区和径流利用区。利用黑河上游控制站莺落峡站和中游控制站正义峡站水文资料，分析了黑河中游耗水量的变化，结果表明：20世纪80年代以前黑河中游耗水量在4．4亿m^3左右，比较稳定；进入20世纪80年代以后，耗水量明显增加，80年代耗水量比70年代以前增加了1．97亿－2.49亿m^3;90年代耗水量又比80年代增加了1.64亿m^3左右；80年代以来流域耗水量呈明显增加的趋势，这是以人类活动影响为主的结果。