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Current understanding of ELF4 deficiency:a novel inborn error of immunity

World Journal of Pediatrics 2024年第5期20卷 444-450页

作者： Hong-Qiang Du Xiao-dong zhao Department of Rheumatology&Immunology National Clinical Research Center for Child Health and DisordersMinistry of Education Key Laboratory of Child Development and DisordersChongqing Key Laboratory of Child Rare Diseases in Infection and ImmunityChildren’s Hospital of Chongqing Medical UniversityZhongshan Er Road 136Yuzhong DistrictChongqingChina

Background ELF4 deficiency has been recently recognized as a novel disorder within the spectrum of inborn errors of immunity(IEIs),specifically categorized as a“disease of immune dysregulation.”Cases of this condition,reported by our team and others,are very limited worldwide.As such,our current knowledge of this new disease remains preliminary.This review aims to provide a brief overview of the clinical manifestations,pathogenesis,and treatment strategies for this novel IEI.Data sources A comprehensive review was conducted after an extensive literature search in the PubMed/Medline database and websites concerning transcriptional factor ELF4 and reports concerning patients with ELF4 deficiency.Our search strategy was“ELF4 OR ETS-related transcription factor Elf-4 OR EL4-like factor 4 OR myeloid Elf-1-like factor”as of the time of manuscript submission.Results The current signature manifestations of ELF4 deficiency disorder are recurrent and prolonged oral ulcer,abdominal pain,and diarrhea in pediatric males.In some cases,immunodeficiency and autoimmunity can also be prominent.Targeted Sanger sequencing or whole exome sequencing can be used to detect variation in ELF4 gene.Western blotting for ELF4 expression of the patient’s cells can confirm the pathogenic effect of the variant.To fully confirm the pathogenicity of the variant,further functional test is strongly advised.Glucocorticoid and biologics are the mainstream management of ELF4 deficiency disorder.Conclusions Pediatric males presenting with recurring ulcerations in digestive tract epithelium with or without recurrent fever should be suspected of DEX.When atypical presentations are prominent,variations in ELF4 gene should be carefully evaluated functionally due to the complex nature of ELF4 function.Experience of treating DEX includes use of glucocorticoid and biologics and more precise treatment needs more patients to identify and further mechanistic study.

关键词： ELF4 transcription factor Immune dysregulation Inborn errors of immunity Mechanism Recurrent infections

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Carbon-coated hybrid crystals with fast electrochemical reaction kinetics for ultra-stable and high-load sodium-ion batteries

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Rare Metals 2024年第2期43卷 647-657页

作者： Chuan Ou Ming-dong Tan Zhen-Bang Li zhao-Jie Li Fei Tian Zheng-Ping Qiao Dan-ni Lei Cheng-Xin Wang State Key Laboratory of Optoelectronic Materials and Technologies School of Materials Science and EngineeringSun Yat-Sen UniversityGuangzhou510275China

Owing to its high theoretical capacity and low cost,Sn has attracted significant attention in sodium-ion batteries.However,the slow kinetics of electrochemical reactions and the rapid decay of capacity resulting from drastic changes in the volume of Sn,as well as persistent side reactions between Sn and the organic electrolyte during the(de)sodium process,have limited its commercialization.To improve the electrochemical performance of Sn-based materials,the bottom-up method is normally used to prepare carbon-coated nanoparticles.However,its complex preparation processes and harsh conditions make it unsuitable for practical applications.Herein,a carbon-coated hybrid crystal composite(Sn/SnO_(x)@C)was prepared using an up-bottom method with commercial Sn/SnO nanoparticles.Various effects accelerate the electrochemical kinetics and inhibit the coarsening of Sn crystals.The Sn/SnO_(x)@C composite electrode exhibited capacity retention of 80.7%even after approximately 1000 cycles(360.4 mAh·g^(−1)) at a current density of 1 A·g^(−1).The high-load Na_(3)V_(2)(PO4)3||Sn/SnO_(x)@C full cell presents a capacity retention rate of 91.7%after 150 cycles at the current density of 0.5 A·g^(−1).This work may significantly accelerate the commercialization of the Sn/SnO_(x)@C composite in sodium-ion batteries with high energy density.

关键词： Sodium-ion batteries(SIBs) Sn-based anode Ultraviolet curing Up-bottom method

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Resilient control of flexible hypersonic vehicles against unmatched distributed faults

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Chinese Journal of Aeronautics 2024年第5期37卷 60-70页

作者： dong zhao Wenjing REN School of Artificial Intelligence Anhui UniversityHefei 230601China Key Laboratory of System Control and Information Processing Ministry of EducationShanghai Jiao Tong UniversityShanghai 200240China School of Electrical Engineering and Automation Hefei University of TechnologyHefei 230009China

This paper studies a robust fault compensation and vibration suppression problem of flexible hypersonic vehicles.The controlled plant is represented by a cascade system composed of a nonlinear Ordinary Differential Equation(ODE)and an Euler-Bernoulli Beam Equation(EBBE),in which the vibration dynamics is coupled with the rigid dynamics and suffers from distributed faults.A state differential transformation is introduced to transfer distributed faults to an EBBE boundary and a longitudinal dynamics is refined by utilizing T-S fuzzy IF-THEN rules.A novel T-S fuzzy based fault-tolerant control algorithm is developed and related stability conditions are established.The robust exponential stability and well-posedness are proved by using the modified l_(0)-semigroup based Lyapunov direct approach.A simulation study on the longitudinal dynamics of flexible hypersonic vehicles effectively verifies the validity of the developed theoretical results.

关键词： Flexible hypersonic vehicles ODE-EBBE cascade Robust exponential stability Unmatched distributed fault compensation Vibration suppression

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Current status of liver transplantation for human immunodeficiency virus-infected patients in China's Mainland

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World Journal of Gastroenterology 2024年第14期30卷 1958-1962页

作者： Jian-Xin Tang dong zhao Department of Liver Surgery and Organ Transplantation Center Shenzhen Third People’s HospitalThe Second Affiliated Hospital of Southern University of Science and TechnologyNational Clinical Research Center for Infectious DiseaseShenzhen 518000Guangdong ProvinceChina

According to the report from the Chinese Center for Disease Control and Prevention,the prevalence of human immunodeficiency virus(HIV)infection exceeded 1.2 million individuals by the year 2022,with an annual increase of about 80000 cases.The overall prevalence of hepatitis B surface antigen among individuals co-infected with HIV reached 13.7%,almost twice the rate of the general population in China.In addition to the well-documented susceptibility to opportunistic infections and new malignancies,HIV infected patients frequently experience liver-related organ damage,with the liver and kidneys being the most commonly affected.This often leads to the development of end-stage liver and kidney diseases.Therefore,organ transplantation has emerged as an important part of active treatment for HIV infected patients.However,the curative effect is not satisfactory.HIV infection has been considered a contraindication for organ transplantation.Until the emergence of highly active anti-retroviral therapy in 1996,the once intractable replication of retrovirus was effectively inhibited.With prolonged survival,the failure of important organs has become the main cause of death among HIV patients.Therefore,transplant centers worldwide have resu-med exploration of organ transplantation for HIV-infected individuals and reached a positive conclusion.This study provides an overview of the current landscape of HIV-positive patients receiving liver transplantation(LT)in main-land China.To date,our transplant center has conducted LT for eight end-stage liver disease patients co-infected with HIV,and all but one,who died two months postoperatively due to sepsis and progressive multi-organ failure,have survived.Comparative analysis with hepatitis B virus-infected patients during the same period revealed no statistically significant differences in acute rejection reactions,cytomegalovirus infection,bacteremia,pulmonary infections,acute kidney injury,new-onset cancers,or vascular and biliary complications.

关键词： Liver transplantation Human immunodeficiency virus Infection Hepatitis B virus End-stage liver disease China'Mainland

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Simplified prediction models for acoustic installation effects of train-mounted equipment

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Railway Engineering Science 2024年第2期32卷 125-143页

作者： David Thompson dong zhao Giacomo Squicciarini Institute of Sound and Vibration Research University of SouthamptonSouthampton SO171BJUK

Acoustic models of railway vehicles in standstill and pass-by conditions can be used as part of a virtual certification process for new trains.For each piece of auxiliary equipment,the sound power measured on a test bench is combined with meas-ured or predicted transfer functions.It is important,however,to allow for installation effects due to shielding by fairings or the train body.In the current work,fast-running analytical models are developed to determine these installation effects.The model for roof-mounted sources takes account of diffraction at the corner of the train body or fairing,using a barrier model.For equipment mounted under the train,the acoustic propagation from the sides of the source is based on free-field Green’s functions.The bottom surfaces are assumed to radiate initially into a cavity under the train,which is modelled with a simple diffuse field approach.The sound emitted from the gaps at the side of the cavity is then assumed to propagate to the receivers according to free-field Green’s functions.Results show good agreement with a 2.5D boundary element model and with measurements.Modelling uncertainty and parametric uncertainty are evaluated.The largest variability occurs due to the height and impedance of the ground,especially for a low receiver.This leads to standard deviations of up to 4 dB at low frequencies.For the roof-mounted sources,uncertainty over the location of the corner used in the equivalent barrier model can also lead to large standard deviations.

关键词： Train noise Auxiliary equipment Acoustic installation effects Virtual certification Uncertainty

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Exercise promotes osteogenic differentiation by activating the long non-coding RNA H19/microRNA-149 axis

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World Journal of Orthopedics 2024年第4期15卷 363-378页

作者： Xu-Chang Zhou dong-Xue Wang Chun-Yu Zhang Ya-Jing Yang Ruo-Bing zhao Sheng-Yao Liu Guo-Xin ni School of Sport Medicine and Rehabilitation Beijing Sport UniversityBeijing 100084China Department of Acupuncture and Moxibustion Hubei University of Chinese MedicineWuhan 430065Hubei ProvinceChina Department of Spinal Surgery The Second Affiliated Hospital of Guangzhou Medical UniversityGuangzhou 510260Guangdong ProvinceChina Department of Rehabilitation Medicine The First Affiliated Hospital of Xiamen UniversityXiamen 361003Fujian ProvinceChina

BACKGROUND Regular physical activity during childhood and adolescence is beneficial to bone development,as evidenced by the ability to increase bone density and peak bone mass by promoting bone formation.AIM To investigate the effects of exercise on bone formation in growing mice and to investigate the underlying mechanisms.METHODS 20 growing mice were randomly divided into two groups:Con group(control group,n=10)and Ex group(treadmill exercise group,n=10).Hematoxylin-eosin staining,immunohistochemistry,and micro-CT scanning were used to assess the bone formation-related indexes of the mouse femur.Bioinformatics analysis was used to find potential miRNAs targets of long non-coding RNA H19(lncRNA H19).RT-qPCR and Western Blot were used to confirm potential miRNA target genes of lncRNA H19 and the role of lncRNA H19 in promoting osteogenic differentiation.RESULTS Compared with the Con group,the expression of bone morphogenetic protein 2 was also significantly increased.The micro-CT results showed that 8 wk moderate-intensity treadmill exercise significantly increased bone mineral density,bone volume fraction,and the number of trabeculae,and decreased trabecular segregation in the femur of mice.Inhibition of lncRNA H19 significantly upregulated the expression of miR-149 and suppressed the expression of markers of osteogenic differentiation.In addition,knockdown of lncRNA H19 significantly downregulated the expression of autophagy markers,which is consistent with the results of autophagy-related protein changes detected in mouse femurs by immunofluorescence.CONCLUSION Appropriate treadmill exercise can effectively stimulate bone formation and promote the increase of bone density and bone volume in growing mice,thus enhancing the peak bone mass of mice.The lncRNA H19/miR-149 axis plays an important regulatory role in osteogenic differentiation.

关键词： Exercise Osteogenic differentiation Bone formation Bone mesenchymal stem cells Autophagy

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High‑Entropy Layered Oxide Cathode Enabling High‑Rate for Solid‑State Sodium‑Ion Batteries

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Nano-Micro Letters 2024年第1期16卷 160-171页

作者： Tianxun Cai Mingzhi Cai Jinxiao Mu Siwei zhao Hui Bi Wei zhao Wujie dong Fuqiang Huang State Key Laboratory of High Performance Ceramics and Superfine Microstructure Shanghai Institute of CeramicsChinese Academy of SciencesShanghai 200050People’s Republic of China State Key Laboratory of Rare Earth Materials Chemistry and Applications College of Chemistry and Molecular EngineeringPeking UniversityBeijing 100871People’s Republic of China Center of Materials Science and Optoelectronics Engineering University of Chinese Academy of SciencesBeijing 100049People’s Republic of China Zhongke Institute of Strategic Emerging Materials Yixing 214213JiangsuPeople’s Republic of China

Na-ion O3-type layered oxides are prospective cathodes for Na-ion batteries due to high energy density and low-cost.Nevertheless,such cathodes usually suffer from phase transitions,sluggish kinetics and air instability,making it difficult to achieve high performance solid-state sodium-ion batteries.Herein,the high-entropy design and Li doping strategy alleviate lattice stress and enhance ionic conductivity,achieving high-rate performance,air stability and electrochemically thermal stability for Na_(0.95)Li_(0.06)ni_(0.25)Cu_(0.05)Fe_(0.15)Mn_(0.49)O_(2).This cathode delivers a high reversible capacity(141 mAh g^(−1)at 0.2C),excellent rate capability(111 mAh g^(−1)at 8C,85 mAh g^(−1)even at 20C),and long-term stability(over 85%capacity retention after 1000 cycles),which is attributed to a rapid and reversible O3–P3 phase transition in regions of low voltage and suppresses phase transition.Moreover,the compound remains unchanged over seven days and keeps thermal stability until 279℃.Remarkably,the polymer solid-state sodium battery assembled by this cathode provides a capacity of 92 mAh g^(−1)at 5C and keeps retention of 96%after 400 cycles.This strategy inspires more rational designs and could be applied to a series of O3 cathodes to improve the performance of solid-state Na-ion batteries.

关键词： High-entropy High-rate performance Li-TM interaction Air stability O3 layered oxide cathode

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Power equipment vibration visualization using intelligent sensing method based on event-sensing principle

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Global Energy Interconnection 2024年第2期7卷 228-240页

作者： Mingzhe zhao Xiaojun Shen Lei Su Zihang dong Tongji University No.1239 Siping RoadYangpu DistrictShanghai 200092P.R.China State Grid Shanghai Municipal Electric Power Company Electric Power Science Research Institute Shanghai 200437P.R.China Department of Electrical and Electronic Engineering Imperial College LondonLondonSW72AZU.K.

Vibration measurements can be used to evaluate the operation status of power equipment and are widely applied in equipment quality inspection and fault identification.Event-sensing technology can sense the change in surface light intensity caused by object vibration and provide a visual description of vibration behavior.Based on the analysis of the principle underlying the transformation of vibration behavior into event flow data by an event sensor,this paper proposes an algorithm to reconstruct event flow data into a relationship correlating vibration displacement and time to extract the amplitude-frequency characteristics of the vibration signal.A vibration measurement test platform is constructed,and feasibility and effectiveness tests are performed for the vibration motor and other power equipment.The results show that event-sensing technology can effectively perceive the surface vibration behavior of power and provide a wide dynamic range.Furthermore,the vibration measurement and visualization algorithm for power equipment constructed using this technology offers high measurement accuracy and efficiency.The results of this study provide a new noncontact and visual method for locating vibrations and performing amplitude-frequency analysis on power equipment.

关键词： Power equipment Event sensing Non contact measurement Graphic display Feasibility

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Prospect Theory Based Individual Irrationality Modelling and Behavior Inducement in Pandemic Control

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Computer Modeling in Engineering & Sciences 2024年第7期140卷 139-170页

作者： Wenxiang dong H.Vicky zhao Department of Automation BNRistTsinghua UniversityBeijing100084China

Understanding and modeling individuals’behaviors during epidemics is crucial for effective epidemic control.However,existing research ignores the impact of users’irrationality on decision-making in the epidemic.Meanwhile,existing disease control methods often assume users’full compliance with measures like mandatory isolation,which does not align with the actual situation.To address these issues,this paper proposes a prospect theorybased framework to model users’decision-making process in epidemics and analyzes how irrationality affects individuals’behaviors and epidemic dynamics.According to the analysis results,irrationality tends to prompt conservative behaviors when the infection risk is low but encourages risk-seeking behaviors when the risk is high.Then,this paper proposes a behavior inducement algorithm to guide individuals’behaviors and control the spread of disease.Simulations and real user tests validate our analysis,and simulation results show that the proposed behavior inducement algorithm can effectively guide individuals’behavior.

关键词： Disease spread behavior model irrationality prospect theory

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Tracking lithiation with transmission electron microscopy

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Science China Chemistry 2024年第1期67卷 291-311页

作者： Xincheng Lei Jianxiong zhao Jiayi Wang dong Su Beijing National Laboratory for Condensed Matter Physics Institute of PhysicsChinese Academy of SciencesBeijing 100190China South China Academy of Advanced Optoelectronics&International Academy of Optoelectronics at zhaoqing South China Normal UniversityGuangzhou 510006China

Li-ion batteries(LIBs)have dominated energy-storage techniques for portable electronic devices and electric cars,and are expanding their territory into the large-scale energy storage.The energy storage of LIBs is realized by the reversible shuttle of lithium ions between electrodes.It is essential to track the lithium diffusion and obtain a profound insight into the lithiation mechanism during the work cycle of LIBs.Transmission electron microscopy(TEM)is a powerful tool for the structural characterization,which can provide the information about the lithiation at the atomic scale.In this review,we summarize the research frontiers of TEM applications on LIBs.We introduce the techniques for the direct observation of Li species in LIB-related materials.Especially,the application of cryo-TEM is highlighted.Moreover,in-situ TEM technique is further discussed since it shows great advantages in studying the dynamical structure changes of LIBs.The perspectives and strategies in this review offer feasible guidance for researchers to further improve the performance of LIBs.

关键词： lithium-ion battery electrode materials transmission electron microscopy cryo-TEM in-situ TEM

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