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伴遗传性RET基因变异的Van Wyk-Grumbach综合征1例

第三届中国卫星导航学术年会

作者： Zhou Xionglin 1 , Liu Bo 2 , Cheng Jian 2 , dong feihong 1 1. Institute of Communication Engineering, PLA University of Science and Technology, Nanjing, China, 210007 2. The Key Lab of Military Satellite Communication Institute of Communication Engineering, PLA University of Science and Technology, Nanjing, China, 210007

BOC modulation has the advantages of low interference with BPSK and high anti-fading capability. The main challenge in BOC tracking is represented by the presence of multiple peaks in the correction function. Various tech- niques have been designed for solving the multiple-peak ambiguity problem for special forms of BOC signals. In this paper, Different algorithms, such as BPSKlike, Bump-Jump, Double Estimate and Unambiguity Discrimination are analyzed and compared. A method based on ASPeCT Technique for baseband signal processing is simulated, which can improve code tracking reliability. Simulation results prove the effect of the method.

关键词： satellite navigation BOC modulation multiple peaks ambiguity synchronization

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中华儿科杂志 2024年第8期62卷 790-792页

作者：董文科王俊祺高广林詹博文孙艳艳罗飞宏俞建复旦大学附属儿科医院中医科上海201102 上海交通大学附属瑞金医院儿科上海200025 复旦大学附属儿科医院内分泌科上海201102

6.4岁女童以甲状腺功能减退、性早熟、肝功能损伤、贫血等多系统异常为主要表现,结合血清学检测和影像学检查,诊断为Van Wyk-Grumbach综合征。经足量左旋甲状腺素替代治疗后,上述症状均明显改善,治疗期间自行药物减量,导致症状反复和线... 详细信息

6.4岁女童以甲状腺功能减退、性早熟、肝功能损伤、贫血等多系统异常为主要表现,结合血清学检测和影像学检查,诊断为Van Wyk-Grumbach综合征。经足量左旋甲状腺素替代治疗后,上述症状均明显改善,治疗期间自行药物减量,导致症状反复和线性生长受损。外周血基因检测发现该患儿携带RET基因杂合变异,来源于患儿母亲。伴遗传性RET基因变异的Van Wyk-Grumbach综合征尚未见报道。

关键词： RET基因肝功能损伤甲状腺功能减退 Van 影像学检查基因检测线性生长血清学检测

Enhanced thermal and frequency stability and decent fatigue endurance in lead-free NaNbO_(3) -based ceramics with high energy storage density and efficiency

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Journal of Materiomics 2022年第2期8卷 489-497页

作者： Hongyun Chen Junpeng Shi Xiaoyan dong feihong Pang Hailin Zhang Xiuli Chen Huanfu Zhou School of Materials Science and Engineering Guilin University of TechnologyGuilin 541004China

Lead-free ceramic capacitors have the application prospect in the dielectric pulse power system due to the advantages of large dielectric constant,lower dielectric loss and good temperature ***-theless,most reported dielectric ceramics have limitation of realizing large energy storage density(W_(rec))and high energy storage efficiency(h)simultaneously due to the low breakdown electric field(E_(b)),low maximum polarization and large remanent polarization(P_(r)).These issues above can be settled by raising the bulk resistivity of dielectric ceramics and optimizing domain ***,we designed a new system by doping(Bi_(0.5)Na_(0.5))_(0.7)Sr_(0.3)TiO_(3) into 0.9NaNbO_(3)-0.1Bi(Ni_(0.5)Zr_(0.5))O_(3) ceramics,which simulta-neously obtained a higher bulk resistivity by decreasing the grain size and achieved a smaller P_(r) by optimizing domain structure,thus the better E_(b) of 530 kV/cm and W_(rec) of 6.43 J/cm^(3) were achieved,h was improved from 34%to 82%.Besides,the 0.4BNST ceramics show excellent temperature,frequency and fatigue stability under the conditions of 20-180℃,1-100 Hz and 104 cycles,***-while,superior power density(P_(D)=107 MW/cm^(3)),large current density(C_(D)=1070 A/cm^(2))and discharge speed(1.025 m s)were achieved in 0.4BNST ***,the charge-discharge performance displayed good temperature stability in the temperature range of 30℃-180℃.The above results indicated that the ceramics have potential practical value in the field of energy storage capacitor.

关键词： Breakdown electric field Excellent dielectric stability Charge-discharge performance Potential value

High energy storage density and power density achieved simultaneously in NaNbO_(3)-based lead-free ceramics via antiferroelectricity enhancement

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Journal of Materiomics 2021年第3期7卷 629-639页

作者： Xiaoyan dong Xu Li Xiuli Chen Hongyun Chen Congcong Sun Junpeng Shi feihong Pang Huanfu Zhou Collaborative Innovation Center for Exploration of Hidden Nonferrous Metal Deposits and Development of New Materials in Guangxi Key Laboratory of Nonferrous Materials and New Processing TechnologyMinistry of EducationSchool of Materials Science and EngineeringGuilin University of TechnologyGuilin541004China

High-performance lead-free dielectric ceramics with simultaneously high energy storage density and power density are in high demanded for pulse power *** realize excellent energy-storage characteristics,a strategy to enhance antiferroelectricity and construct a local random field simultaneously was proposed in this *** on the above strategy,a series of(1-x)NaNbO_(3)-xBi(Ni_(1/2)Sn_(1/2))O3[xBNS,x=0.05,0.10,0.15,0.20,and 0.22]solid solutions were designed and *** ultrahigh energy storage density(Utotal)of 7.35 J/cm^(3),and recoverable energy density(Urec)of 5.00 J/cm^(3) were achieved in the 0.10BNS *** addition,an adequate stability of energy storage properties at a range of temperatures(20e140℃),frequencies(1e100 Hz),and fatigue test durations(1e1-10^(4) cycles)were realized in 0.10BNS ceramics.0.10BNS ceramics displayed a high current density of 1005 A/cm2,an ultrahigh power density of 100.5 MW/cm^(3,)and an ultrashort discharge time of 46.5 ns?This remarkable performance not only justified our strategy but also confirmed 0.10BNS ceramics as a promising candidate for energy storage.

关键词： Lead-free ceramics Energy storage NaNbO_(3)-Based Power density

Self-supported porous heterostructure WC/WO_(3−x)ceramic electrode for hydrogen evolution reaction in acidic and alkaline media

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Journal of Advanced Ceramics 2022年第8期11卷 1208-1221页

作者： feihong WANG Binbin dong Junwei WANG Nianwang KE Chuntian TAN Anding HUANG Yutong WU Luyuan HAO Liangjun YIN Xin XU Yuxi XIAN Simeon AGATHOPOULOS CAS Key Laboratory of Materials for Energy Conversion Department of Materials Science and EngineeringUniversity of Science and Technology of ChinaHefei 230026China School of Materials Science and Engineering Henan Key Laboratory of Special Protective MaterialsLuoyang Institute of Science and TechnologyLuoyang 471023China Yangtze Delta Region Institute(Huzhou) University of Electronic Science and Technology of ChinaHuzhou 313001China School of Energy Science and Engineering University of Electronic Science and Technology of ChinaChengdu 611731China CAS Key Laboratory of Mechanical Behaviors and Design of Materials Department of Modern MechanicsUniversity of Science and Technology of ChinaHefei 230026China Department of Materials Science and Engineering University of IoanninaIoannina GR-45110Greece

Tungsten carbide(WC)-based materials are widely considered as the hydrogen evolution reaction(HER)process catalysts due to their“Pt-like”electronic ***,traditional powder electrodes have a high cost,and display problems related to the process itself and the poor stability over operation *** paper presented a self-supported asymmetric porous ceramic electrode with WO_(3-x)whiskers formed in situ on the walls of the finger-like holes and membrane surface,which was prepared by combining phase inversion tape-casting,pressureless sintering,and thermal treatment in a CO_(2) *** optimized ceramic electrode displayed good catalytic HER activity and outstanding stability at high current *** specifically,it demonstrated the lowest overpotentials of 107 and 123 mV and the lowest Tafel slopes of 59.3 and 72.4 mV·dec^(-1)at 10 mA·cm^(-2)in acidic and alkaline media,*** superior performance was ascribed to the structure of the ceramic membrane and the charge transfer efficiency,which was favored by the in situ developed WC/WO_(3-x)heterostructure and the oxygen vacancies.

关键词： hydrogen evolution reaction(HER) porous ceramic membrane WC/WO_(3−x)heterostructure self-supported electrode oxygen vacancies

性别认同与性别表达的社会困境:医务社工介入跨性别儿童青少年多学科联合诊疗案例研究

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中国医学伦理学 2024年第7期37卷 843-851页

作者：董颖刘泳志罗飞宏魏佳师正楠孔祥宁郝宇昕杨宁郑章乾傅丽丽国家儿童医学中心/复旦大学附属儿科医院社会工作部上海201102 国家儿童医学中心/复旦大学附属儿科医院内分泌遗传代谢科上海201102 国家儿童医学中心/复旦大学附属儿科医院精神心理科上海201102 上海大学社会学院上海200444 复旦大学社会发展与公共政策学院上海200433 华东理工大学社会与公共管理学院上海200237

跨性别儿童青少年(跨儿)在性别认同和表达过程中面临严重的社会困境,阻碍该群体寻求合理及平等的生存发展权利。在平权视角及社会困境理论框架下,通过对在某院跨性别多学科(MDT)门诊寻求医务社工帮助的跨儿进行访谈,揭示出在传统“二元... 详细信息

跨性别儿童青少年(跨儿)在性别认同和表达过程中面临严重的社会困境,阻碍该群体寻求合理及平等的生存发展权利。在平权视角及社会困境理论框架下,通过对在某院跨性别多学科(MDT)门诊寻求医务社工帮助的跨儿进行访谈,揭示出在传统“二元性别”体制下,跨儿社会包容性及基础设施不足,导致该群体在性别表达和性别认同过程中同时遭遇“社会陷阱”和“社会藩篱”两大社会困境。具体表现为,跨儿的社会性别选择常导致集体非理性反应和性别惩罚,影响其获取合法有效的诊疗服务。为此,研究团队运用批判性方法论,构建医务社工参与的跨儿多学科联合诊疗路径,并验证该路径在合理化跨儿及其家庭自身需求、缓解其在性别认同过程中的心理压力和社会适应问题、培养所在家庭多元对话环境、提升自我效能和社会参与度等方面有显著干预效果,以期为社会困境中的跨儿群体提供帮助,助力其权益纳入儿童友好指标体系,提升全社会对跨儿群体的包容和理解。

关键词：跨性别儿童青少年性伦理社会困境多学科联合诊疗

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无接触网供电城轨车辆中人体电磁场评估

电工技术学报 2021年第S1期36卷 40-45页

作者：董亮林飞宏王晨曲张秋敏西南交通大学电气工程学院成都611756 中车唐山机车车辆有限公司唐山063035

该文采用有限元法对曝露于大功率无接触网供电城轨车辆中的人体进行电磁安全评估,以检测该无接触网供电城轨车辆是否低于人体电磁曝露安全值。以500kW无接触网供电城轨车辆为例,通过数值仿真计算车内漏磁水平,并对车内佩戴心脏起搏器的... 详细信息

该文采用有限元法对曝露于大功率无接触网供电城轨车辆中的人体进行电磁安全评估,以检测该无接触网供电城轨车辆是否低于人体电磁曝露安全值。以500kW无接触网供电城轨车辆为例,通过数值仿真计算车内漏磁水平,并对车内佩戴心脏起搏器的人体进行感应电磁场和比吸收率(SAR)评估,将计算结果与国际非电离辐射防护委员会(ICNIRP)中规定的限值进行比较。结果表明,人体内部感应电场较低,但是在人体表面感应出较高电场,装有心脏起搏器的组织SAR值远低于2W/kg的限值,人体站在渡板上方时,脚踝处SAR值最高并且十分接近局部SAR限值。这些结果对于无接触网供电技术在轨道交通领域的安全应用起到了积极的作用。

关键词：无接触网供电电磁曝露比吸收率感应电场心脏起搏器

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无接触网供电车辆电磁-热场耦合计算

西南交通大学学报 2020年第3期55卷 545-551页

作者：王艳琴张秋敏林飞宏董亮中车唐山机车车辆有限公司河北唐山063035 西南交通大学电气工程学院四川成都611756

无接触网供电技术在城市轨道交通中的应用受到越来越广泛的关注,大功率无接触电能传输的实际应用还有许多问题需要论证,其中电磁感应导致的涡流发热问题引起人们的普遍重视.依据电磁感应原理和传热学,建立无接触网供电车辆感应加热模型... 详细信息

无接触网供电技术在城市轨道交通中的应用受到越来越广泛的关注,大功率无接触电能传输的实际应用还有许多问题需要论证,其中电磁感应导致的涡流发热问题引起人们的普遍重视.依据电磁感应原理和传热学,建立无接触网供电车辆感应加热模型,采用有限元法计算无接触网供电车辆热场分布,对不同载荷工况下的车辆的发热情况进行数值仿真,并对采用散热器和风冷两种散热方式的接收线圈的散热性能进行对比研究.研究结果表明:接收线圈和转向架温度升高明显;随着发射线圈电流增加以及气隙距离的减小,车辆各个部位的温度都有上升趋势;装有散热器的接收线圈最高温度比不含散热器时降低了126.0℃,通过改变散热器的传热系数能进一步提高散热器的散热性能;采用风冷散热方式接收线圈温度降低了131.2℃,与散热器相比,风冷的散热性能略好,且随着风速增加风冷效果更加突出.

关键词：无接触网供电车辆电磁-热场耦合温度场有限元法

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无线传能机车人体电磁环境安全研究

电工技术学报 2022年第11期37卷 2665-2672页

作者：杨威徐英雷王晨曲林飞宏董亮西南交通大学电气工程学院成都610031

随着无线电能传输技术在铁路系统的应用,电磁安全问题引起越来越多人的关注。为了有效评估乘客及司机在无线传能机车内的电磁安全问题,该文利用三维电磁仿真软件构建无线传能系统辐射下车内人员电磁环境模型,研究处于车厢内不同位置的... 详细信息

随着无线电能传输技术在铁路系统的应用,电磁安全问题引起越来越多人的关注。为了有效评估乘客及司机在无线传能机车内的电磁安全问题,该文利用三维电磁仿真软件构建无线传能系统辐射下车内人员电磁环境模型,研究处于车厢内不同位置的司机和三名乘客(乘客A、乘客B、小孩)暴露的磁场强度、电场强度和电流密度的分布情况。结果表明:位于司机室的司机感应电场强度最小,其感应电场强度值为2×10^(-5)V/m;乘客A感应电场强度为最大,其最大值达到0.55V/m;乘客B所处的车厢连接风挡处磁场强度最大,其最大值为2.78A/m;感应电流密度最大值出现在乘客B的腹部位置,其最大值为13.7μA/m^(2)。所有计算结果均低于国际非电离辐射防护委员会(ICNIRP)导则的电磁场限值,说明位于车内的司机和乘客都是安全的。最后通过现场测试得到的数据与数值计算结果对比,结果较为吻合,进一步验证了仿真的正确性。

关键词：机车无线传能感应电场电磁暴露安全评估