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Mental retardation,seizures and language delay caused by new SETD1B mutations:Three case reports

World Journal of Clinical Cases 2024年第2期12卷 383-391页

作者： Le Ding li-wan Wei Tai-song li Jing Chen Department of Neurology Children’s Hospital of Nanjing Medical UniversityNanjing 210000Jiangsu ProvinceChina Chigene(Beijing)Translational Medical Research Center Co.Ltd.Beijing 101111China

BACKGROUND The SETD1B gene is instrumental in human intelligence and nerve development.Mutations in the SETD1B gene have been linked in recent studies to neurodevelopmental disorders,seizures,and language delay.CASE SUMMARY This study aimed to analyze the clinical manifestations and treatment of three patients suffering from mental retardation,epilepsy,and language delay resulting from a new mutation in the SETD1B gene.Three individuals with these symptoms were selected,and their clinical symptoms,gene test results,and treatment were analyzed.This article discusses the impact of the SETD1B gene mutation on patients and outlines the treatment approach.Among the three patients(two females and one male,aged 8,4,and 1,respectively),all exhibited psychomotor retardation,attention deficit,and hyperactivity disorder,and two had epilepsy.Antiepileptic treatment with sodium tripolyvalproate halted the seizures in the affected child,although mental development remained somewhat delayed.Whole exome sequencing revealed new mutations in the SETD1B gene for all patients,specifically with c.5473C>T(p.Arg1825trp),c.4120C>T(p.Gln1374*,593),c.14_15insC(p.His5Hisfs*33).CONCLUSION Possessing the SETD1B gene mutation may cause mental retardation accompanied by seizures and language delay.Although the exact mechanism is not fully understood,interventions such as drug therapy,rehabilitation training,and family support can assist patients in managing their symptoms and enhancing their quality of life.Furthermore,genetic testing supplies healthcare providers with more precise diagnostic and therapeutic guidance,informs families about genetic disease risks,and contributes to understanding disease pathogenesis and drug research and development.

关键词： Neurodevelopmental disorder Seizure SETD1B gene Whole-exome sequencing New mutation Case report

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Topological magnetoelectric response in ferromagnetic axion insulators

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National Science Review 2024年第2期11卷 8-14页

作者： Yuhao wan Jiayu li Qihang liu Department of Physics and Shenzhen Institute for Quantum Science and Engineering(SIQSE) Southern University of Science and Technology Shenzhen Key Laboratory of Advanced Quantum Functional Materials and Devices Southern University of Science and Technology Guangdong Provincial Key Laboratory for Computational Science and Material Design Southern University of Science and Technology

The topological magnetoelectric effect （TME） is a hallmark response of the topological field theory,which provides a paradigm shift in the study of emergent topological phenomena.However,its direct observation is yet to be realized due to the demanding magnetic configuration required to gap all surface states.Here,we theoretically propose that axion insulators with a simple ferromagnetic configuration,such as the MnBi2Te4/（Bi2Te3）nfamily,provide an ideal playground to realize the TME.In the designed triangular prism geometry,all the surface states are magnetically gapped.Under a vertical electric field,the surface Hall currents give rise to a nearly half-quantized orbital moment,accompanied by a gapless chiral hinge mode circulating in parallel.Thus,the orbital magnetization from the two topological origins can be easily distinguished by reversing the electric field.Our work paves the way for direct observation of the TME in realistic axion-insulator materials.

关键词： topological magnetoelectric effect axion insulator magnetic topological materials

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Double-layered skeleton of li alloy anchored on 3D metal foam enabling ultralong lifespan of li anode under high rate

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Chinese Chemical Letters 2024年第3期35卷 479-485页

作者： Chaohui Wei Zeyu Yao Jin Ruan Zhicui song Aijun Zhou Yingze song Donghuang wang Jicheng Jiang Xin wang Jingze li Yangtze Delta Region Institute(Huzhou) University of Electronic Science and Technology of ChinaHuzhou 313000China School of Materials and Energy University of Electronic Science and Technology of ChinaChengdu 611731China State Key Laboratory of Environmental-Friendly Energy Materials Tianfu Institute of Research and InnovationSchool of Materials and ChemistrySouthwest University of Science and TechnologyMianyang 621010China

The high specific capacity and low negative electrochemical potential of lithium metal anodes(LMAs),may allow the energy density threshold of li metal batteries(LMBs)to be pushed higher.However,the existing detrimental issues,such as dendritic growth and volume expansion,have hindered the practical implementation of LMBs.Introducing three-dimensional frameworks(e.g.,copper and nickel foam),have been regarded as one of the fundamental strategies to reduce the local current density,aiming to extend the Sand'time.Nevertheless,the local environment far from the skeleton is almost the same as the typical plane li,due to macroporous space of metal foam.Herein,we built a double-layered 3D current collector of li alloy anchored on the metal foam,with micropores interconnected macropores,via a viable thermal infiltration and cooling strategy.Due to the excellent electronic and ionic conductivity coupled with favorable lithiophilicity,the li alloy can effectively reduce the nucleation barrier and enhance the li^(+)transportation rate,while the metal foam can role as the primary promotor to enlarge the surface area and buffer the dimensional variation.Synergistically,the li composite anode with hierarchical structure of primary and secondary scaffolds realized the even deposition behavior and minimum volume expansion,outputting preeminent prolonged cycling performances under high rate.

关键词： Double-layered skeleton li alloy 3D Metal foam Ultralong lifespan lithium metal batteries High rate

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Predictors of Adverse Pregnancy Outcomes Following Traumatic Injuries

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Current Medical Science 2024年第3期44卷 642-647页

作者： wan-rong LU Ping WU Gong song Mei-qi GU Zhe XU li HE National Clinical Research Center for Gynecology and Obstetrics Cancer Biology Research CenterTongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan430030China Department of Traumatic Surgery Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan430030China

Objective After traumatic injury in pregnant women,providing timely and appropriate management for high-risk patients is crucial for both pregnant women and fetuses.This study aimed to identify risk factors that predict adverse pregnancy outcomes after traumatic injury.Methods A retrospective cohort study including 317 pregnant patients who experienced trauma was conducted.The collected data included general demographics,injury mechanisms and adverse pregnancy outcomes.Patients were divided into two subgroups based on the absence or presence of trauma-related adverse pregnancy outcomes.Univariate and multivariate logistic regressions were conducted to estimate the associations between clinical variables and adverse pregnancy outcomes.Results A total of 41(12.93%)patients experienced adverse pregnancy outcomes within the first 24 h post-trauma.This study revealed that age>35 years(OR=14.995,95%CI:5.024–44.755,Plity of posttraumatic adverse pregnancy outcomes.Maternal age,gestational age at delivery,vaginal bleeding and positive FAST results were risk factors for abnormal delivery.Conclusion Advanced maternal age,third trimester,and positive FAST results should alert multidisciplinary trauma teams to closely monitor patients to prevent adverse pregnancy outcomes.

关键词： adverse pregnancy outcomes predictive factors abnormal delivery trauma

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Vibration Control of A Flexible Marine Riser System Subject to Input Dead Zone and Extraneous Disturbances

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China Ocean Engineering 2024年第2期38卷 271-284页

作者： ZHOU li wanG Guo-rong wan Min School of Mechatronic Engineering Southwest Petroleum UniversityChengdu 610500China Energy Equipment Institute Southwest Petroleum UniversityChengdu 610500China National Key Laboratory of Oil and Gas Reservoir Geology and Exploitation Southwest Petroleum UniversityChengdu 610500China

An observer-based adaptive backstepping boundary control is proposed for vibration control of flexible offshore riser systems with unknown nonlinear input dead zone and uncertain environmental disturbances.The control algorithm can update the control law online through real-time data to make the controller adapt to the environment and improve the control precision.Specifically,based on the adaptive backstepping framework,virtual control laws and Lyapunov functions are designed for each subsystem.Three direction interference observers are designed to track the timevarying boundary disturbance.On this basis,the inverse of the dead zone and linear state transformation are used to compensate for the original system and eliminate the adverse effects of the dead zone.In addition,the stability of the closed-loop system is proven by Lyapunov stability theory.All the system states are bounded,and the vibration offset of the riser converges to a small area of the initial position.Finally,four examples of flexible marine risers are simulated in MATLAB to verify the effectiveness of the proposed controller.

关键词： adaptive backstepping control disturbance observer flexible marine riser input dead zone vibration control

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A defective iron-based perovskite cathode for high-performance IT-SOFCs:Tailoring the oxygen vacancies using Nb/Ta co-doping

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Journal of Energy Chemistry 2024年第1期88卷 306-316,I0008页

作者： Bayu Admasu Beshiwork Xinyu wan Min Xu Haoran Guo Birkneh Sirak Teketel Yu Chen Jun song Chen Tingshuai li Enrico Traversa School of Materials and Energy University of Electronic Science and Technology of ChinaChengdu 611731SichuanChina The Future Laboratory Tsinghua UniversityBeijing 100084China School of Chemical Sciences University of Chinese Academy of SciencesBeijing 100049China Schoolof Environment and Energy South China University of Technlogy Guangzhou 50006GuangongChina Institute for Advanced Study Chengdu UniversityChengdu 610106SichuanChina

The sluggish kinetics of the electrochemical oxygen reduction reaction(ORR)in intermediatetemperature solid oxide fuel cells(IT-SOFCs)greatly limits the overall cell performance.In this study,an efficient and durable cathode material for IT-SOFCs is designed based on density functional theory(DFT)calculations by co-doping with Nb and Ta the B-site of the SrFeO_(3-δ)perovskite oxide.The DFT calculations suggest that Nb/Ta co-doping can regulate the energy band of the parent SrFeO_(3-δ)and help electron transfer.In symmetrical cells,such cathode with a SrFe_(0.8)Nb_(0.1)Ta_(0.1)O_(3-δ)(SFNT)detailed formula achieves a low cathode polarization resistance of 0.147Ωcm^(2) at 650℃.Electron spin resonance(ESR)and X-ray photoelectron spectroscopy(XPS)analysis confirm that the co-doping of Nb/Ta in SrFeO_(3-δ)B-site increases the balanced concentration of oxygen vacancies,enhancing the electrochemical performance when compared to 20 mol%Nb single-doped perovskite oxide.The cathode button cell with NiSDC|SDC|SFNT configuration achieves an outstanding peak power density of 1.3 W cm^(-2)at 650℃.Moreover,the button cell shows durability for 110 h under 0.65 V at 600℃ using wet H_(2) as fuel.

关键词： Solid oxide fuel cell Cathode Oxygen reduction reaction Power density DFT calculation

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CBA: multi source fusion model for fast and intelligent target intention identification

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Journal of Systems Engineering and Electronics 2024年第2期35卷 406-416页

作者： wan Shichang li Qingshan wanG Xuhua LU Nanhua School of Computer Science and Technology Xidian UniversityXi’an 710071China School of Mathematics and Computer Application Shangluo UniversityShangluo 726000China

How to mine valuable information from massive multisource heterogeneous data and identify the intention of aerial targets is a major research focus at present. Aiming at the longterm dependence of air target intention recognition, this paper deeply explores the potential attribute features from the spatiotemporal sequence data of the target. First, we build an intelligent dynamic intention recognition framework, including a series of specific processes such as data source, data preprocessing,target space-time, convolutional neural networks-bidirectional gated recurrent unit-atteneion (CBA) model and intention recognition. Then, we analyze and reason the designed CBA model in detail. Finally, through comparison and analysis with other recognition model experiments, our proposed method can effectively improve the accuracy of air target intention recognition,and is of significance to the commanders’ operational command and situation prediction.

关键词： intention massive data deep network artificial intelligence

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Functional Optical Fiber Sensors Detecting Imperceptible Physical/Chemical Changes for Smart Batteries

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Nano-Micro Letters 2024年第8期16卷 268-308页

作者： Yiding li li wang Youzhi song Wenwei wang Cheng lin Xiangming He Institute of Nuclear and New Energy Technology Tsinghua UniversityBeijing 100084People’s Republic of China National Engineering Research Center of Electric Vehicles Beijing Institute of Technology(BIT)Beijing 100081People’s Republic of China Shenzhen Automotive Research Institute of BIT(Shenzhen Research Institute of National Engineering Research Center of Electric Vehicles) Shenzhen 518118People’s Republic of China

The battery technology progress has been a contradictory process in which performance improvement and hidden risks coexist.Now the battery is still a“black box”,thus requiring a deep understanding of its internal state.The battery should“sense its internal physical/chemical conditions”,which puts strict requirements on embedded sensing parts.This paper summarizes the application of advanced optical fiber sensors in lithium-ion batteries and energy storage technologies that may be mass deployed,focuses on the insights of advanced optical fiber sensors into the processes of one-dimensional nano-micro-level battery material structural phase transition,electrolyte degradation,electrode-electrolyte interface dynamics to three-dimensional macro-safety evolution.The paper contributes to understanding how to use optical fiber sensors to achieve“real”and“embedded”monitoring.Through the inherent advantages of the advanced optical fiber sensor,it helps clarify the battery internal state and reaction mechanism,aiding in the establishment of more detailed models.These advancements can promote the development of smart batteries,with significant importance lying in essentially promoting the improvement of system consistency.Furthermore,with the help of smart batteries in the future,the importance of consistency can be weakened or even eliminated.The application of advanced optical fiber sensors helps comprehensively improve the battery quality,reliability,and life.

关键词： Smart battery Advanced embedded optical fiber sensor Battery internal physical/chemical state Quality-reliability-life characteristic

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Multi-Time-Scale Resource Allocation Based on Long-Term Contracts and Real-Time Rental Business Models for Shared Energy Storage Systems

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Journal of Modern Power Systems and Clean Energy 2024年第2期12卷 454-465页

作者： Yuxuan Zhuang Zhiyi li Qipeng Tan Yongqi li Minhui wan College of Electrical Engineering Zhejiang UniversityHangzhouChina China Southern Power Grid Power Generation Company Energy Storage Research Institute GuangzhouChina IEEE

The push for renewable energy emphasizes the need for energy storage systems(ESSs)to mitigate the unpre-dictability and variability of these sources,yet challenges such as high investment costs,sporadic utilization,and demand mismatch hinder their broader adoption.In response,shared energy storage systems(SESSs)offer a more cohesive and efficient use of ESS,providing more accessible and cost-effective energy storage solutions to overcome these obstacles.To enhance the profitability of SESSs,this paper designs a multi-time-scale resource allocation strategy based on long-term contracts and real-time rental business models.We initially construct a life cycle cost model for SESS and introduce a method to estimate the degradation costs of multiple battery groups by cycling numbers and depth of discharge within the SESS.Subsequently,we design various long-term contracts from both capacity and energy perspectives,establishing associated models and real-time rental models.Lastly,multi-time-scale resource allocation based on the decomposition of user demand is proposed.Numerical analysis validates that the business model based on long-term contracts excels over models operating solely in the real-time market in economic viability and user satisfaction,effectively reducing battery degradation,and leveraging the aggregation effect for SESS can generate an additional increase of 10.7%in net revenue.

关键词： Capacity allocation long-term contracts shared energy storage system stochastic programming

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Plastic deformation behavior of a Cu-10Ta alloy under strong impact loading

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Defence Technology（防务技术） 2024年第2期32卷 368-382页

作者： Ping song Jianghai liu Wenbin li Yiming li Research Institute of Chemical Defense Academy of Military ScienceBeijing102205China Ministerial Key Laboratory of ZNDY Nanjing University of Science and TechnologyNanjing210094China

In this work,a Cu-10Ta alloy with a copper to tantalum mass ratio of 9:1 is prepared using powder metallurgy technology.Physical properties of the alloy,including density,microstructure,melting point,and constant-volume specific heat,are tested.Via the split Hopkinson pressure bar(SHPB)and flyerplate impact experiments,the relationship between equivalent stress and equivalent plastic strain of the material is studied at temperatures of 298-823 K and under strain rates of 1×10^(-3)-5.2×10^(3)s^(-1),and the Hugoniot relationship at impact pressures of 1.46-17.25 GPa and impact velocities of 108-942 m/s is obtained.Evolution of the Cu-10Ta microstructure that occurs during high-strain-rate impact is analyzed.Results indicate that the Cu-10Ta alloy possesses good thermal stability,and at ambient temperatures of up to 50%its melting point,stress softening of less than 15%of the initial strength is observed.A modified J-C constitutive model is employed to accurately predict the variation of yield strength with strain rate.Under strong impact,the copper phase is identified as the primary source of plastic deformation in the Cu-10Ta alloy,while significant deformation of the high-strength tantalum particles is less pronounced.Furthermore,the longitudinal wave speed D is found to correlate linearly with the particle velocity u upon strong impact.Analysis reveals that the sound speed and spallation strength of the alloy increase with increasing impact pressure.

关键词： Cu-10Ta SHPB Plastic deformation Flyer impact Hugoniot relationship

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