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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 *** 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

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Experimental and numerical investigation of the impacts of rotor tip-rake on excitation forces of pump-jet propulsors

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Journal of Hydrodynamics 2024年第1期36卷 142-157页

作者： Xue-qin Ji xiao-song Zhang Chen-jun Yang xiao-qian dong State Key Laboratory of Ocean Engineering Marine Numerical Experiment CentreSchool of Naval ArchitectureOcean and Civil EngineeringShanghai Jiao Tong UniversityShanghai200240China CSSC Shanghai Marine Energy Saving Technology Co. Ltd.Shanghai200011China China Ship Scientific Research Center Shanghai Branch Shanghai200011China

The tip-clearance flow in a pump-jet propulsor exerts great impacts on the fluctuating pressures and resultant unsteady forces,which are important sources of structural vibrations and radiated noise *** blade geometry close to the tip is an important factor determining the vortex strength in the tip-clearance *** the open-water condition,the effects of raking the rotor tips on the duct-surface fluctuating pressures and the resultant unsteady forces acting on different components of the propulsor are investigated via physical model experiments and the numerical solution of Reynolds-averaged Navier-Stokes(RANS)equations coupled with the SST k-ωturbulence *** measured and simulated results of hydrodynamic pressures are consistent to each other,and the simulated flows help better understand why the fluctuating pressures change with the tip *** strong fluctuations of duct-surface pressures are caused by intensive tip separation *** duct-surface pressure fluctuations are effectively reduced by using the rake distribution near the tip towards blade back side and,for the combination of the five-bladed rotor and the seven-bladed stator,the resultant unsteady horizontal(and vertical)forces acting on the duct and stator are also reduced;while increasing rake leads to negative effect on pressure fluctuations and unsteady horizontal(and vertical)forces acting on all the components of the propulsor.

关键词： Pump-jet tip-clearance tip-rake fluctuating pressures excitation forces

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New clinical trial design in precision medicine:discovery,development and direction

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Signal Transduction and Targeted Therapy 2024年第4期9卷 1293-1321页

作者： xiao-Peng Duan Bao-dong Qin xiao-dong Jiao Ke Liu Zhan Wang Yuan-Sheng Zang Department of Medical Oncology Changzheng HospitalNaval Medical UniversityShanghaiChina

In the era of precision medicine,it has been increasingly recognized that individuals with a certain disease are complex and different from each *** to the underestimation of the significant heterogeneity across participants in traditional"one-sizefits-all"trials,patient-centered trials that could provide optimal therapy customization to individuals with specific biomarkers were developed including the basket,umbrella,and platform trial designs under the master protocol *** recent years,the successive FDA approval of indications based on biomarker-guided master protocol designs has demonstrated that these new clinical trials are ushering in tremendous *** the rapid increase in the number of basket,umbrella,and platform trials,the current clinical and research understanding of these new trial designs,as compared with traditional trial designs,remains *** majority of the research focuses on methodologies,and there is a lack of in-depth insight concerning the underlying biological logic of these new clinical trial ***,we provide this comprehensive review of the discovery and development of basket,umbrella,and platform trials and their underlying logic from the perspective of precision ***,we discuss future directions on the potential development of these new clinical design in view of the"Precision Pro","Dynamic Precision",and"Intelligent Precision".This review would assist trial-related researchers to enhance the innovation and feasibility of clinical trial designs by expounding the underlying logic,which be essential to accelerate the progression of precision medicine.

关键词： clinical approval successive

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Observation of gapless corner modes of photonic crystal slabs in synthetic translation dimensions

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Photonics Research 2024年第3期12卷 444-455页

作者： WEN-JIN ZHANG HAO-CHANG MO WEN-JIE CHEN xiao-dong CHEN JIAN-WEN dong School of Physics&State Key Laboratory of Optoelectronic Materials and Technologies Sun Yat-sen UniversityGuangzhou 510275China

Second-order topological photonic crystals support localized corner modes that deviate from the conventional bulk-edge ***,the frequency shift of corner modes spanning the photonic band gap has not been experimentally ***,we observe the gapless corner modes of photonic crystal slabs within a parameter space by considering translation as an additional synthetic *** corner modes,protected by topological pumping in synthetic translation dimensions,are found to exist independently of the specific corner *** gapless corner modes are experimentally imaged via the near-field scanning measurement and validated numerically by full-wave *** propose a topological rainbow with gradient translation,demonstrating the ability to extract and separate specific frequency components of light into different spatial *** work contributes to the advancement of topological photonics and provides valuable insights into the exploration of gapless corner modes in synthetic dimensions.

关键词： translation dimensions corner

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Quantitatively Evaluating the Erosion and Preservation of Supergene Oxide Zones:Evidence from the Yulong Porphyry Cu Deposit,Eastern Tibetan Plateau

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Journal of Earth Science 2024年第1期35卷 280-282页

作者： xiao-dong Deng Jian-Wei Li State Key Laboratory of Geological Processes and Mineral Resources and School of Earth ResourcesChina University of GeosciencesWuhan 430074China

*** and erosion of sulfide ore deposits are important for understanding the post-mineralization processes that have significant effects on the preservation of primary orebodies(Velasco et al.,2013;Braxton et al.,2009;Brimhall et al.,1985).Supergene oxide zones are often developed in sulfide ore deposits,and are mainly composed of iron oxides and hydroxides zones in weathering profile(Andrew,1984;Thornber et al.,1981).

关键词： Eastern Tibetan weathering

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The extraction of effective components and an antioxidant activity study of Tulipa edulis

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Food Science and Human Wellness 2024年第1期13卷 276-286页

作者： Doudou Zhang dong xiao Tingting Yin Shuangzhi Zhao Olena Zhur Xun xiao Hailun He Leilei Chen Beijing Advanced Innovation Center for Food Nutrition and Human Health Beijing Technology and Business UniversityBeijing 102488China School of Life Sciences Central South UniversityChangsha 410013China State Key Laboratory of Coal Resources and Safe Mining University of Mining and TechnologyXuzhou 221116China Institute of Agro-Food Science and Technology Shandong Academy of Agricultural SciencesJinan 250100China

Plant extracts from natural sources are an excellent choice for food additives and natural *** this study,the active components of Tulipa edulis were extracted and analysed,and their antioxidant capacity was ***,the crude extract mixture was separated and purified using a Sephadex LH-20 gel,and the antioxidant activity of the purified products was *** umbilical vein endothelial human umbilical vein endothelial cells(HUVEC)cells were treated with 35 mmol/L glucose to construct a model of oxidative ***,the cells were treated with the active component to observe whether the products of *** could have a good protective effect on HUVEC cells induced by *** analysis was also performed on HUVEC cells after same treatment to explore the possible mechanism of the component F2 protecting HUVEC cells from oxidative stress induced by high *** results showed that component F2 obtained from *** has strong antioxidant ***,F2 can play a strong antioxidant protective role in HUVEC ***,the gene expression of heme oxygenase 1(HO-1),γ-glutamyl cysteine ligase catalytic subunit(GCLC)and NAD(P)H quinone oxidoreductase-1(NQO1)in HUVEC cells was up-regulated after treated with *** study provides reference value for the further development and application of *** and the d evelopment of functional food.

关键词： Tulipa edulis Oxidative stress Purification Antioxidant

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Research on the Dynamic Compressibility of Polyurethane Microcellular Elastomer and its Application for Impact Resistance

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Chinese Journal of Polymer Science 2024年第8期42卷 1185-1197页

作者： Zhi-Ying Zhao Hao Jiang xiao-dong Li Xu-dong Zhang Xing Su Mei-Shuai Zou School of Materials Science and Engineering Beijing Institute of TechnologyBeijing 100081China

The packaging materials with cushioning performance are used to prevent the internal contents from being damaged by the impact and vibration of external *** polyurethane microcellular elastomers(PUMEs)can absorb energy through cell collapse and molecular chain *** this study,PUMEs with different densities were investigated by scanning electron microscopy,dynamic mechanical analysis and dynamic compression *** exhibited significant im pact resistance and the maximum peak stress attenuation ratio reached 73.33%.The protective equipment was made by PUME with the optimal density of 600 kg/m^(3),and then the acceleration sensing device installed with the same protective equipment fell from a height of 3,5 and 10 m to evaluate the energy-absorbing property and reusability of *** results showed that PUMEs equipment reduced the peak acceleration of the device by 93.84%,with a maximum deviation of 9%between actual test and simulation,and shortened the impact time of first landing by 57.39%.In addition,the equipment PUMEs equipment could effectively reduce the stress on the protected items.

关键词： Polyurethane microcellular elastomer Impact velocity Stress attenuation ratio Impact resistance

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Working condition recognition of sucker rod pumping system based on 4-segment time-frequency signature matrix and deep learning

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Petroleum Science 2024年第1期21卷 641-653页

作者： Yun-Peng He Hai-Bo Cheng Peng Zeng Chuan-Zhi Zang Qing-Wei dong Guang-Xi Wan xiao-Ting dong State Key Laboratory of Robotics Shenyang Institute of AutomationChinese Academy of SciencesShenyang 110016LiaoningChina Key Laboratory of Networked Control Systems Chinese Academy of SciencesShenyang 110016LiaoningChina Institutes for Robotics and Intelligent Manufacturing Chinese Academy of SciencesShenyang 110169LiaoningChina University of Chinese Academy of Sciences Beijing100049China School of Artifcial Intelligence Shenyang University of TechnologyShenyang 110870LiaoningChina

High-precision and real-time diagnosis of sucker rod pumping system(SRPS)is important for quickly mastering oil well *** learning-based method for classifying the dynamometer card(DC)of oil wells is an efficient diagnosis ***,the input of the DC as a two-dimensional image into the deep learning framework suffers from low feature utilization and high computational ***,different SRPSs in an oil field have various system parameters,and the same SRPS generates different DCs at different ***,there is heterogeneity in field data,which can dramatically impair the diagnostic *** solve the above problems,a working condition recognition method based on 4-segment time-frequency signature matrix(4S-TFSM)and deep learning is presented in this ***,the 4-segment time-frequency signature(4S-TFS)method that can reduce the computing power requirements is proposed for feature extraction of DC ***,the 4S-TFSM is constructed by relative normalization and matrix calculation to synthesize the features of multiple data and solve the problem of data ***,a convolutional neural network(CNN),one of the deep learning frameworks,is used to determine the functioning conditions based on the *** on field data verify that the proposed diagnostic method based on 4S-TFSM and CNN(4S-TFSM-CNN)can significantly improve the accuracy of working condition recognition with lower computational *** the best of our knowledge,this is the first work to discuss the effect of data heterogeneity on the working condition recognition performance of SRPS.

关键词： Sucker-rod pumping system Dynamometer card Working condition recognition Deep learning Time-frequency signature Time-frequency signature matrix

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Screening for metabolic dysfunction-associated fatty liver disease:Time to discard the emperor’s clothes of normal liver enzymes?

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World Journal of Gastroenterology 2024年第22期30卷 2839-2842页

作者： Chen-xiao Huang xiao-dong Zhou Calvin Q Pan Ming-Hua Zheng Metabolic Dysfunction-Associated Fatty Liver Disease Research Center Department of HepatologyThe First Affiliated Hospital of Wenzhou Medical UniversityWenzhou 325000Zhejiang ProvinceChina Department of Cardiovascular Medicine The Key Laboratory of Cardiovascular Diseases of WenzhouThe First Affiliated Hospital of Wenzhou Medical UniversityWenzhou 325000Zhejiang ProvinceChina Division of Gastroenterology and Hepatology Department of MedicineNew York University Langone HealthNew York University Grossman School of MedicineNew YorkNY 11355United States Institute of Hepatology Wenzhou Medical UniversityKey Laboratory of Diagnosis and Treatment for the Development of Chronic Liver Disease in Zhejiang ProvinceWenzhou 325000Zhejiang ProvinceChina

Metabolic dysfunction-associated fatty liver disease(MAFLD)is the most prevalent chronic liver condition *** liver enzyme-based screening methods have limitations that may missed diagnoses and treatment *** Chen et al,the risk of developing MAFLD remains elevated even when alanine aminotransferase levels fall within the normal ***,there is an urgent need for advanced diagnostic techniques and updated algorithms to enhance the accuracy of MAFLD diagnosis and enable early *** paper proposes two potential screening methods for identifying individuals who may be at risk of developing MAFLD:Lowering these thresholds and promoting the use of noninvasive liver fibrosis scores.

关键词： Metabolic dysfunction-associated fatty liver disease Non-alcoholic fatty liver disease Alanine aminotransferase Liver enzymes Screening Noninvasive liver fibrosis scores

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Introducing electrolytic electrochemical polymerization for constructing protective layers on Ni-rich cathodes of Li-ion batteries

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Rare Metals 2024年第6期43卷 2536-2545页

作者： Lin-Tao Dou Bei Li Hao-Long Nie dong-dong xiao Chao-Qun Shang xiao-Min Wang Zhan-Hui Zhang Katerina E.Aifantis Pu Hu School of Materials Science and Engineering Wuhan Institute of TechnologyWuhan 430205China Huairou Division Institute of PhysicsChinese Academy of SciencesBeijing 101400China Department of Mechanical and Aerospace Engineering University of FloridaGainesvilleFL 32603USA

LiNi_(0.8)Co_(0.1)Mn_(0.1)O_(2)(NCM811)is the most promising cathode for high-energy Li-ion batteries,despite its poor cycling stability that originates from the reactions that occur with the ***,to solve this interfacial issue,a facile electrolytic electrochemical polymerization process was introduced in this paper,and a uniform conductive electrolyte interface(polyaniline)was successfully constructed on the surface of the NCM811 porous electrode(PANI-NCM),which facilitated the charge transfer during charge/*** side reactions at the interface between the cathode and the electrolyte are suppressed,and thereby,the cycling performance and rate capability are considerably ***-NCM delivers an initial capacity of 157.2 mAh·g^(-1)as well as excellent cyclability(capacity retention of 88%after 500 cycles at 2C),whereas the capacity of the bare NCM811 has dropped to 31.3 mAh·g^(-1).In addition,polypyrrole and polythiophene also can be formed through electrolytic electrochemical polymerization process,which provides a practicable tactic to modify the interfacial stability of cathodes for high-energy Li-ion batteries.

关键词： Lithium-ion battery Ni-rich cathode In-situ polymerization Conductive polymer Electrochemical performance

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