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

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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 *** such,our current knowledge of this new disease remains *** review aims to provide a brief overview of the clinical manifestations,pathogenesis,and treatment strategies for this novel *** 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 *** 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 *** The current signature manifestations of ELF4 deficiency disorder are recurrent and prolonged oral ulcer,abdominal pain,and diarrhea in pediatric *** some cases,immunodeficiency and autoimmunity can also be *** Sanger sequencing or whole exome sequencing can be used to detect variation in ELF4 *** blotting for ELF4 expression of the patient’s cells can confirm the pathogenic effect of the *** fully confirm the pathogenicity of the variant,further functional test is strongly *** and biologics are the mainstream management of ELF4 deficiency *** Pediatric males presenting with recurring ulcerations in digestive tract epithelium with or without recurrent fever should be suspected of *** atypical presentations are prominent,variations in ELF4 gene should be carefully evaluated functionally due to the complex nature of ELF4 *** 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

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 *** each piece of auxiliary equipment,the sound power measured on a test bench is combined with meas-ured or predicted transfer *** is important,however,to allow for installation effects due to shielding by fairings or the train *** the current work,fast-running analytical models are developed to determine these installation *** model for roof-mounted sources takes account of diffraction at the corner of the train body or fairing,using a barrier *** equipment mounted under the train,the acoustic propagation from the sides of the source is based on free-field Green’s *** bottom surfaces are assumed to radiate initially into a cavity under the train,which is modelled with a simple diffuse field *** 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 *** show good agreement with a 2.5D boundary element model and with *** uncertainty and parametric uncertainty are *** largest variability occurs due to the height and impedance of the ground,especially for a low *** leads to standard deviations of up to 4 dB at low *** 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

Ultrahard X-ray multifunctional application beamline at the SSRF

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Nuclear Science and Techniques 2024年第6期35卷 14-25页

作者： Ke Yang zhao-Hui dong Chun-Yin Zhou Zi-Long zhao dong-Xu Liang Sai-Chao Cao Ai-Guo Li Shanghai Advanced Research Institute Chinese Academy of SciencesShanghai 201210China

The ultrahard X-ray multifunctional application beamline(BL12SW)is a phase-II beamline project at the Shanghai Syn-chrotron Radiation *** primary X-ray techniques used at the beamline are high-energy X-ray diffraction and imaging using white and monochromatic *** main scientific objectives of ultrahard X-ray beamlines are focused on two research *** is the study of the structural properties of Earth’s interior and new materials under extreme high-temperature and high-pressure conditions,and the other is the characterization of materials and processes in near-real service *** beamline utilizes a superconducting wiggler as the light source,with two diamond windows and SiC discs to filter out low-energy light(primarily below 30 keV)and a Cu filter assembly to control the thermal load entering the subsequent optical *** beamline is equipped with dual *** first was a meridional bending Laue monochromator cooled by liquid nitrogen,achieving a full-energy coverage of 30-162 *** second was a sagittal bending Laue monochromator installed in an external building,providing a focused beam in the horizontal direction with an energy range of 60-120 *** were four experimental hutches:two large-volume press experimental hutches(LVP1 and LVP2)and two engineering material experimental hutches(ENG1 and ENG2).Each hutch was equipped with various near-real service conditions to satisfy different *** example,LVP1 and LVP2 were equipped with a 200-ton DDIA press and a 2000-ton dual-mode(DDIA and Kawai)press,***1 and ENG2 provide in situ tensile,creep,and fatigue tests as well as high-temperature *** June 2023,the BL12SW has been in trial *** is expected to officially open to users by early 2024.

关键词： Shanghai Synchrotron Radiation Facility Ultrahard X-ray High energy diffraction High energy imaging Engineering materials Earth science

Ergodicity of 3D Stochastic Burgers Equation

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Acta Mathematica Sinica,English Series 2024年第2期40卷 498-510页

作者： zhao dong Jiang Lun WU Guo Li ZHOU RCSDS Academy of Mathematics and Systems ScienceChinese Academy of SciencesBeijing 100190P.R.China Department of Mathematics Computational FoundrySwansea UniversitySwansea SA18ENUK College of Mathematics and Statistics Chongqing UniversityChongqing 400044P.R.China

3D Burgers equation is an important model for *** is natural to expect the long-time behaviour for this hydrodynamics ***,there is no result about the long-time behaviour for this deterministic ***,if the system is perturbed by stochastic noise,we establish the existence and uniqueness of invariant measure for 3D stochastic Burgers equation.

关键词： 3D stochastic Burgers equations maximum principle ergodicity

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 *** 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 *** 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 *** often leads to the development of end-stage liver and kidney ***,organ transplantation has emerged as an important part of active treatment for HIV infected ***,the curative effect is not *** infection has been considered a contraindication for organ *** the emergence of highly active anti-retroviral therapy in 1996,the once intractable replication of retrovirus was effectively *** prolonged survival,the failure of important organs has become the main cause of death among HIV ***,transplant centers worldwide have resu-med exploration of organ transplantation for HIV-infected individuals and reached a positive *** study provides an overview of the current landscape of HIV-positive patients receiving liver transplantation(LT)in main-land *** 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 *** 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 co

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

Understanding Discrepancy of Power System Dynamic Security Assessment with Unknown Faults: A Reliable Transfer Learning-based Method

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CSEE Journal of Power and Energy Systems 2024年第1期10卷 427-431页

作者： Chao Ren Han Yu Yan Xu zhao Yang dong School of Computer Science and Engineering Nanyang Technological UniversitySingapore School of Electrical and Electronic Engineering Nanyang Technological UniversitySingapore

This letter proposes a reliable transfer learning(RTL)method for pre-fault dynamic security assessment(DSA)in power systems to improve DSA performance in the presence of potentially related unknown *** takes individual discrepancies into consideration and can handle unknown faults with incomplete *** experiment results demonstrate high DSA accuracy and computational efficiency of the proposed RTL *** analysis shows RTL can guarantee system performance.

关键词： Adversarial training dynamic security assessment maximum classifier discrepancy missing data transfer learning

Origin of off-centering effect and the influence on heat transport in thermoelectrics

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Materials Futures 2024年第1期3卷 142-149页

作者： Hongyao Xie Li-dong zhao School of Materials Science and Engineering Beihang UniversityBeijing 100191People’s Republic of China

Recently,off-centering behavior has been discovered in a series of thermoelectric *** behavior indicates that the constituent atoms of the lattice displace from their coordination centers,leading to the locally distorted state and local symmetry breaking,while the material still retains its original crystallographic *** effect has been proved to be the root cause of ultralow thermal conductivity in off-centering materials,and is considered as an effective tool to regulate the thermal conductivity and improve the thermoelectric ***,we present a collection of recently discovered off-centering compounds,discuss their electronic origins and local coordination structures,and illuminate the underlying mechanism of the off-centering effect on phonon transport and thermal *** paper presents a comprehensive view of our current understanding to the off-centering effect,and provides a new idea for designing high performance thermoelectrics.

关键词： thermoelectric thermal conductivity off-centering behavior acoustic-optical phonon scattering

Application of CFD and FEA Coupling to Predict Structural Dynamic Responses of A Trimaran in Uni-and Bi-Directional Waves

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China Ocean Engineering 2024年第1期38卷 81-92页

作者： LIAO Xi-yu XIA Jin-song CHEN Zhan-yang TANG Qin zhao Nan zhao Wei-dong GUI Hong-bin Marine Design&Research Institute of China Shanghai 200011China Ship Structure Division China Ship Scientific Research CenterWuxi 214082China College of Ocean Engineering Harbin Institute of TechnologyWeihaiWeihai 264209China State Key Laboratory of Structural Analysis Optimization and CAE Software for Industrial EquipmentDalian University of TechnologyDalian 116024China School of Foreign Studies Jiangnan UniversityWuxi 214122China

To predict the wave loads of a flexible trimaran in different wave fields,a one-way interaction numerical simulation method is proposed by integrating the fluid solver(Star-CCM+)and structural solver(Abaqus).Differing from the existing coupled CFD-FEA method for monohull ships in head waves,the presented method equates the mass and stiffness of the whole ship to the hull shell so that any transverse and longitudinal section stress of the hull in oblique waves can be ***,verification study and sensitivity analysis are carried out by comparing the trimaran motions using different mesh sizes and time step *** on the wave elevation of uni-and bi-directional waves is also carried *** a comprehensive analysis on the structural responses of the trimaran in different uni-directional regular wave and bi-directional cross sea conditions is carried out,***,the differences in structural response characteristics of trimaran in different wave fields are *** results show that the present method can reduce the computational burden of the two-way fluid-structure interaction simulations.

关键词： CFD-FEA fluid-structure coupling structural responses trimaran bi-directional cross sea

Gaussian Backbone-Based Spherical Evolutionary Algorithm with Cross-search for Engineering Problems

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Journal of Bionic Engineering 2024年第2期21卷 1055-1091页

作者： Yupeng Li dong zhao Ali Asghar Heidari Shuihua Wang Huiling Chen Yudong Zhang College of Computer Science and Technology Changchun Normal UniversityChangchun130032JilinChina School of Surveying and Geospatial Engineering College of EngineeringUniversity of TehranTehranIran School of Computing and Mathematical Sciences University of LeicesterLeicesterLE17RHUK Department of Biological Sciences Xi’an Jiaotong-Liverpool UniversitySuzhou215123JiangsuChina Key Laboratory of Intelligent Informatics for Safety and Emergency of Zhejiang Province Wenzhou UniversityWenzhou325035China School of Computer Science and Engineering Southeast UniversityNanjing210096China

In recent years,with the increasing demand for social production,engineering design problems have gradually become more and more *** novel and well-performing meta-heuristic algorithms have been studied and developed to cope with this *** them,the Spherical Evolutionary Algorithm(SE)is one of the classical representative methods that proposed in recent years with admirable optimization ***,it tends to stagnate prematurely to local optima in solving some specific ***,this paper proposes an SE variant integrating the Cross-search Mutation(CSM)and Gaussian Backbone Strategy(GBS),called *** this study,the CSM can enhance its social learning ability,which strengthens the utilization rate of SE on effective information;the GBS cooperates with the original rules of SE to further improve the convergence effect of *** objectively demonstrate the core advantages of CGSE,this paper designs a series of global optimization experiments based on IEEE CEC2017,and CGSE is used to solve six engineering design problems with *** final experimental results fully showcase that,compared with the existing well-known methods,CGSE has a very significant competitive advantage in global tasks and has certain practical value in real ***,the proposed CGSE is a promising and first-rate algorithm with good potential strength in the field of engineering design.

关键词： Meta-heuristic algorithms Engineering optimization Spherical evolution algorithm Global optimization