架空输电线路环境荷载响应与设计理论的研究

我国地域广阔, 气候跨区域变化较大, 地形地貌复杂多变, 各地区风、雨以及导线覆冰的分布规律千差万别。由于目前环境荷载因素对输电塔线结构的严重破坏频繁发生, 造成了巨大的经济损失。因此, 有必要建立输电塔线结构环境荷载响应分析理论和进行设计优化, 提高其结构设计的规范水准, 并加强灾害监测控制。这对减轻环境荷载造成的输电塔线结构的灾害具有不可估量的作用。     

强电场作用下早龄期混凝土的氯离子迁移特征

电化学除氯技术能有效降低混凝土结构中的有害氯离子,降低混凝土结构失效风险。采用强电场方式对混凝土结构进行电化学除氯,并在不同的早龄期时间介入40、80、120 min的强电场作用,测试了内部氯离子含量变化和分布特征,并对通电后养护28 d试件进行回弹强度试验。研究结果表明：早龄期介入强电场作用能显著提高除氯效率,达到相同除氯效果时的作业时间约为传统电化学方法的1/300;氯离子分布特征表明,自由氯离子分布较传统电化学参数作用时更均匀,结合氯离子仍然存在保护层中间堆积现象;强电场作用后进行标准养护,回弹测试结果表明,强电场对混凝土的保护层宏观强度无明显影响。     

论泾河东庄水库灰岩坝址渗漏问题

通过对泾河东庄水库灰岩坝址工程地质钻探成果特别是钻孔压水试验及水文地质成果包括区域水质、水位、水温等不连通性的分析研究,并结合流量测定三维渗漏数值模拟分析,对坝区渗漏问题进行分析和论证,进一步证明在泾河下游东庄灰岩段建坝是可行的,不会发生严重渗漏,为项目的设计、审查、决策方面参考,以促进工程前期工作的进展。     

引红济石调水工程地质特点及应对措施

陕西引红济石调水工程地质条件非常复杂,施工过程中多次出现断层破碎带塌方、突发性涌水、涌泥等地质难题。结合施工采用的方法、措施分析、研究地质特性和规律,总结施工过程中发生地质灾害的经验教训,认为可以通过加强施工过程控制来有效降低隧洞施工地质风险和灾害发生的可能,从而达到加快工程建设进度,节约工程投资的目的。     

基于成组技术的电机制造CAPP系统

根据电机厂产品的特殊性及电机零件结构特点,自行设计了具有一定针对性的13位分类编码系统,通过工艺设计标准化、自动化过程,成功开发一套适合电机生产特点的DJ-1CAPP系统,解决电机生产过程中工艺设计的瓶颈问题。     

DInSAR监测地表三维形变的方法

本文介绍了几种利用DInSAR技术监测地表三维形变的方法,并深入分析了它们各自的优缺点,对它们的使用条件和使用中的应注意的事项进行了讨论。对于DInSAR监测地表三维形变方法的选择,应取决于数据条件,地质情况以及精度等几个因素。文章的最后展望了DInSAR监测地表三维形变方法的发展趋势。     

一类具高阶非线性项的发展方程的准确周期解

本文导出了具高阶非线性项的Lienard方程的准确周期解并从理论上给予了证明,然后利用这些公式得到一大批具高阶非线性项的发展方程的各种Jacobi椭圆函数型的准确周期解,由此避免了一大类非线性发展方程求周期解时求解过程的重复。     

Deep Phenotyping of Superficial Epidermolytic Ichthyosis due to a Recurrent Mutation in KRT2

Superficial epidermolytic ichthyosis (SEI) is an autosomal dominant inherited ichthyosis. SEI is caused by mutations in KRT2 and frequently shows erythroderma and widespread blistering at birth. We report the clinical manifestations of two patients from a Japanese family with SEI caused by a hotspot mutation, p.Glu487Lys, in KRT2. In addition, we summarize previous reports on SEI patients with the identical mutation. One of the two patients had disease onset at the age of 7 months. The other patient’s age of onset is unknown, but it was in childhood. Neither of the two patients showed erythroderma. To perform deep phenotyping, we studied the age of onset and the frequency of erythroderma in 34 reported SEI cases with the p.Glu487Lys mutation, including the present cases. Among the cases with sufficient clinical information, 44.4% of the cases that were due to p.Glu487Lys in KRT2 occurred at birth. Erythroderma was observed in 11.1% of the cases with p.Glu487Lys in KRT2.     

Systematic Review: Drug Repositioning for Congenital Disorders of Glycosylation (CDG)

Advances in research have boosted therapy development for congenital disorders of glycosylation (CDG), a group of rare genetic disorders affecting protein and lipid glycosylation and glycosylphosphatidylinositol anchor biosynthesis. The (re)use of known drugs for novel medical purposes, known as drug repositioning, is growing for both common and rare disorders. The latest innovation concerns the rational search for repositioned molecules which also benefits from artificial intelligence (AI). Compared to traditional methods, drug repositioning accelerates the overall drug discovery process while saving costs. This is particularly valuable for rare diseases. AI tools have proven their worth in diagnosis, in disease classification and characterization, and ultimately in therapy discovery in rare diseases. The availability of biomarkers and reliable disease models is critical for research and development of new drugs, especially for rare and heterogeneous diseases such as CDG. This work reviews the literature related to repositioned drugs for CDG, discovered by serendipity or through a systemic approach. Recent advances in biomarkers and disease models are also outlined as well as stakeholders’ views on AI for therapy discovery in CDG.     

矩形窄缝通道内流动不稳定起始点计算模型评价

以未除气去离子水为工质,在P=1～15 MPa,G=500～2 000 kg/(m2 · s),ΔTsub,in=20～100 ℃,q=40～1 000 kW/m2的参数范围,以1 000 mm×25 mm×2 mm矩形窄缝通道内垂直向上流动条件下流动不稳定起始点的实验数据为依据,对Saha-Zuber,Levy,Bowring等人提出的预测OSV点关系式和Whittle & Forgan,Lee & Bankoff,Kennedy等人提出的预测OFI点关系式进行了对比分析.结果表明:建立在过冷沸腾基础上的这些预测关系式对OFI点的预测偏差大部分在±20%以内,而其预测结果在低热流密度下低于实验值,在高热流密度下预测结果高于实验值.同时,基于实验数据建立了一个流动不稳定起始点的计算关系式:qOFI=1.95,其预测偏差在±15%以内.与其它实验数据的对比结果表明:本文得到的关系式对其它通道也具有比较好的适用性.