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Next‑Generation Sequencing‑Based Copy Number Variation Analysis in Chinese Patients with Primary Ciliary Dyskinesia Revealed Novel DNAH5 Copy Number Variations

Phenomics

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Phenomics 2024年第1期4卷 24-33页

作者： weicheng Chen Zhuoyao Guo Mengru Li wei sheng Guoying Huang Cardiovascular Center Children’s Hospital of Fudan University399 Wanyuan RoadShanghai 201102China Respirology Department Children’s Hospital of Fudan UniversityShanghai 201102China

Primary ciliary dyskinesia(PCD)is a rare disorder characterized by extensive genetic heterogeneity.However,in the genetic pathogenesis of PCD,copy number variation(CNV)has not received sufcient attention and has rarely been reported,especially in China.Next-generation sequencing(NGS)followed by targeted CNV analysis was used in patients highly suspected to have PCD with negative results in routine whole-exome sequencing(WES)analysis.Quantitative real-time polymerase chain reaction(qPCR)and Sanger sequencing were used to confrm these CNVs.To further characterize the ciliary phenotypes,high-speed video microscopy analysis(HSVA),transmission electron microscopy(TEM),and immunofuorescence(IF)analysis were used.Patient 1(F1:II-1),a 0.6-year-old girl,came from a nonconsanguineous family-I.She presented with situs inversus totalis,neonatal respiratory distress,and sinusitis.The nasal nitric oxide level was markedly reduced.The respiratory cilia beat with reduced amplitude.TEM revealed shortened outer dynein arms(ODA)of cilia.chr5:13717907-13722661del spanning exons 71–72 was identifed by NGS-based CNV analysis.Patient 2(F2:IV-4),a 37-year-old man,and his eldest brother Patient 3(F2:IV-2)came from a consanguineous family-II.Both had sinusitis,bronchiectasis and situs inversus totalis.The respiratory cilia of Patient 2 and Patient 3 were found to be uniformly immotile,with ODA defects.Two novel homozygous deletions chr5:13720087_13733030delinsGTTTTC and chr5:13649539_13707643del,spanning exons 69–71 and exons 77–79 were identifed by NGS-based CNV analysis.Abnormalities in DNA copy number were confrmed by qPCR amplifcation.IF showed that the respiratory cilia of Patient 1 and Patient 2 were defcient in dynein axonemal heavy chain 5(DNAH5)protein expression.This report identifed three novel DNAH5 disease-associated variants by WES-based CNV analysis.Our study expands the genetic spectrum of PCD with DNAH5 in the Chinese population.

关键词： Primary ciliary dyskinesia DNAH5 gene Copy number variation Phenotypic heterogeneity

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Theoretical study on the stability of nanobubbles and its verification in molecular dynamics simulation

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Particuology 2024年第4期87卷 99-105页

作者： sheng Mi Chaofeng Hou wei Ge School of Chemical Engineering and Technology Tianjin UniversityTianjin300072China State Key Laboratory of Multiphase Complex Systems Institute of Process EngineeringChinese Academy of SciencesBeijing.100190China School of Chemical Engineering University of Chinese Academy of SciencesBeijing100049China

The stability of vapor nanobubbles in bulk liquid was investigated theoretically and the critical bubble size was derived from macroscale thermodynamic equations,below which the system destabilizes with sharp drop in pressure.This critical size was quantitatively verified in molecular dynamic simulation using the Lennard-Jones model of argon,where stronger attraction between the molecules at lower density is found to contribute most to the drop of system pressure and,as the Laplace pressure on the curved bubble interface fails to balance the pressure difference across the interface,the bubbles become unstable.The theoretical model could be extended to other systems where reliable equations of state and interfacial tension are available.

关键词： Nanobubble Stability Phase diagram Critical size

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H∞ Robustness for Distributed Control in Autonomous Microgrids Considering Cyber Disturbances

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CSEE Journal of Power and Energy Systems 2024年第2期10卷 696-706页

作者： Ge Cao Guannan Lou wei Gu Lina sheng School of Electrical Engineering Southeast UniversityNanjing 210096China

The physical connections and logical relationships between microgrids and communication networks allow microgrids to develop into typical cyber-physical systems(CPSs).With the extensive use of open communication mechanisms,the impact of cyber disturbances in public communication networks cannot be diminshed.In this paper,a parameter optimal method for a distributed secondary controller based on the robust control theory and consensus algorithm is presented to enhance the robustness of a secondary control system under data disturbance,parameter perturbation,and time delay.First,a distributed secondary control strategy of microgrids is demonstrated that coordinates frequency and voltage restoration and power sharing.Then,considering the impact of cyber events on the secondary control,a distributed robust controller gain design method is proposed to satisfy the H∞ performance index.The solution of the distributed robust control is transformed into a linear matrix in equation problem and latency margin is simultaneously obtained.Finally,a test microgrid CPS is simulated with and without time delay to investigate the impact of cyber events on system operational states and the effectiveness and robustness of the proposed method.

关键词： Cyber disturbance cyber-physical lsystem distributed control microgrid robust H∞control

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Transient Stability Analysis and Design of VSGs with Different DC-Link Voltage Controllers

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CSEE Journal of Power and Energy Systems 2024年第2期10卷 593-604页

作者： Chao Shen wei Gu Wanxing sheng Keyan Liu School of Electrical Engineering Southeast UniversityNanjingChina Power Distribution Research Department China Electric Power Research InstituteBeijing 100192China

Virtual synchronous control has been widely studied for the advantages of emulating inertia for voltage source converters (VSCs). A constant dc-link voltage is usually assumed in existing literature to estimate transient stability of virtual synchronous generators (VSGs). However, actual power supply in the dc-side of VSGs is limited and different dc-link voltage controllers are needed to achieve power balance between DC side and AC side. Addition of dc-link voltage controller has great influence on transient behavior of VSGs, which has not been investigated by previous research. To fill this gap, this paper gives insights into the effect of dc-link voltage dynamics on transient stability of VSGs. First, two typical kinds of VSGs with dc-link voltage controllers are introduced. Then, mathematical models considering dc-link dynamics are established and the effect of dc-link voltage controllers on transient synchronization stability of VSGs is revealed through equal area criterion (EAC). It is found that dc-link voltage controller would reduce stability margin of VSGs and design-oriented transient stability analysis is carried out quantitively using critical clearing time (CCT). Finally, simulation results are given to validate correctness of theoretical analysis.

关键词： DC-link voltage dynamics equal area criterion(eac) transient synchronization stability virtual synchronous generator(VSG)

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Source model of the 2018 MW7.5 Papua New Guinea earthquake constrained by InSAR measurements: Insight into a blind rupture beneath the New Guinea Highlands

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Geodesy and Geodynamics 2024年第4期15卷 315-325页

作者： wei Chen Tianchen sheng wei Xiong Key Laboratory of Earthquake Geodesy Institute of SeismologyChina Earthquake Administration Qinghai Provincial Basic Surveying and Mapping Institute School of Geodesy and Geomatics Wuhan University

The Southern Highland Fold and Thrust Belt（SHFTB）, the boundary of the Australian plate and the New Guinea Highland block, significantly contributes to the convergent deformation along the plate boundary. However, due to the lack of observation data, the detailed slip pattern of the SHFTB and the orogenic mechanism beneath the New Guinea Highlands remains controversial. On 25 February 2018, the MW7.5 Papua New Guinea（PNG） earthquake struck the southeastern segment of the SHFTB. The detailed rupture characteristics of this event is significant for further clarifying the inter-seismic slip pattern along the SHFTB. Here, the coseismic deformation field of this earthquake was obtained using high-resolution ALOS-2 satellite images. We find that the 2018 MW7.5 PNG earthquake ruptured a large-scaled fault（SHFTB） extending to the lower crust（deeper than 20 km） beneath the New Guinea Highlands, with a dip angle of 24°. The slips on the fault plane are equivalent to moment magnitudes of MW7.51. Three major asperities with thrust-dominated slip of up to 3.94 m are detected on the fault plane. This finding implies that the slip pattern on the eastern segment of the SHFTB is dominated by thrust, rather than with significant sinistral movement, as previously reported. The tectonic deformation across the New Guinea Highlands is possibly concentrated on the large-scale fault SHFTB and primarily controls the intracontinental orogeny in the central Papua New Guinea.

关键词： 2018 M W 7.5 Papua New Guinea earthquake The Southern Highlands Fold-and-Thrust Belt InSAR Slip distribution Seismic risk

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High Speed Sintering:Assessing the Influence of Energy Input on Microstructure and Mechanical Properties of Polyether Block Amide(PEBA)Parts

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Chinese Journal of Polymer Science 2024年第5期42卷 675-682,I0004,I0010页

作者： Jiang-Tao Sun Zhi-Yong Fan Yi-wei Mao wei Li wei Zhu Dao-sheng Cai Qing-Song wei State Key Laboratory of Materials Processing and Die&Mould Technology Huazhong University of Science and TechnologyWuhan430074China State Key Laboratory of Refractories and Metallurgy Wuhan University of Science and TechnologyWuhan430074China National Engineering Research Centre for High Efficiency Grinding Hunan UniversityChangsha410082China Wuhan EasyMFG Technology CO. LTDWuhan430074China

High speed sintering,a new powder-bed fusion additive manufacturing technology,utilizes infrared lights(IR)to intensely heat and melt polymer powders.The presence of defects such as porosity,which is associated with particle coalescence,is highly dependdent on the level of energy input.This study investigate the influcence of energy input on porosity and its subsequent effects on the mechanical properties and microstructures of PEBA parts.The parts were manufactured with a variety of lamp powers,resulting in a range of energy input levels spanning from low to high.Subsequebtly,they underwent testing using Archimedes’method,followed by tensile testing.The porosity,mechanical characteristics,and energy input exhibit a strong correlation;inadequate energy input was the primary cause of pore formation.Using the reduced IR light power resulted in the following outcomes:porosity,ultimate tensile strength,and elongation of 1.37%,7.6 MPa,and 194.2%,respectively.When the energy input was further increased,the porosity was reduced to as low as 0.05%and the ultimate tensile strength and elongation were increased to their peak values of 233.8%and 9.1 MPa,respectively.

关键词： High speed sintering Additive manufacturing Energy input Powder bed fusion PEBA

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Experimental,Numerical,and Analytical Studies on the Bending of Mechanically Lined Pipe

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China Ocean Engineering 2024年第2期38卷 221-232页

作者： wei Wen-bin YUAN Lin ZHOU Jia-sheng LIU Zheng State Key Laboratory of Hydraulic Engineering Simulation and Safety Tianjin UniversityTianjin 300072China School of Civil Engineering Tianjin UniversityTianjin 300072China School of Mechanical Engineering Tianjin UniversityTianjin 300072China

Mechanically lined pipe(MLP)is often used for offshore oil and gas transport because of its low cost and corrosion resistance.During installation and operation,the pipe may undergo severe bending deformation,which causes the liner to separate from the outer pipe and buckles,affecting the stability of the whole line.In this paper,the buckling response of MLP subjected to bending is investigated to clarify its bending characteristics by employing both experiments,numerical simulation,as theoretical methods.Two types of MLPs were manufactured with GB 45 carbon steel(SLP)and Al 6061(ALP)used as the outer pipe material,respectively.The hydraulic expansion and bending experiments of small-scale MLPs are conducted.In addition to the ovalized shape of the cross-section for the SLP specimens,the copper liner was found to wrinkle on the compressive side.In contrast,the liner of ALP remains intact without developing any wrinkling and collapse mode.In addition,a dedicated numerical framework and theoretical models were also established.It was found both the manufacturing and bending responses of the MLP can be well reproduced,and the predicted maximum moment and critical curvatures are in good agreement with the experimental results.

关键词： lined pipe bending nonlinear ring theory buckling plasticity

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Hydrodynamic characteristics and particle tracking of 90° lateral intakes at an inclined river slope

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Water Science and Engineering 2024年第2期17卷 197-208页

作者： wei He Si-yuan Feng Jian Zhang Hong-wu Tang Yang Xiao sheng Chen Chun-sheng Liu College of Water Conservancy and Hydropower Engineering Hohai UniversityNanjing 210098China State Key Laboratory of Hydrology Water Resources and Hydraulic Engineering Hohai UniversityNanjing 210098China Collaborative Innovation Center on Water Safety and Water Science Hohai UniversityNanjing 210098China

Lateral intakes are common in rivers.The pump effciency and sediment deposition are determined by the local hydrodynamic characteristics and mainstream division width.The hydraulic characteristics of lateral withdrawal from inclined river slopes at different intake elevations should be investigated.Meanwhile,the division width exhibits significant vertical non-uniformity at an inclined river slope,which should be clarified.Hence,a three-dimensional(3-D)hydrodynamic and particle-tracking model was developed with the Open Source Field Operation and Manipulation(Open FOAM),and the model was validated with physical model tests for 90°lateral withdrawal from an inclined side bank.The flow fields,withdrawal sources,and division widths were investigated with different intake bottom elevations,withdrawal discharges,and main channel velocities.This study showed that under inclined side bank conditions,water entered the intake at an oblique angle,causing significant 3-D spiral flows in the intake rather than two-dimensional closed recirculation.A lower withdrawal discharge,a lower bottom elevation of the intake,or a higher main channel velocity could further strengthen this phenomenon.The average division width and turbulent kinetic energy were smaller under inclined side bank conditions than under vertical bank conditions.With a low intake bottom elevation,a low withdrawal discharge,or a high main channel velocity,the sources of lateral withdrawal were in similar ranges near the local inclined bank in the vertical direction.Under inclined slope conditions,sediment deposition near the intake entrance could be reduced,compared to that under vertical slope conditions.The results provide hydrodynamic and sediment references for engineering designs for natural rivers with inclined terrains.

关键词： Lateral withdrawal Hydrodynamic characteristics Particle tracking Inclined river slope Bottom elevation of intake OpenFOAM

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Development of a prognostic scoring system for hepatic vena cava Budd-Chiari syndrome with hepatocellular carcinoma

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Hepatobiliary & Pancreatic Diseases International 2024年第4期23卷 370-375页

作者： sheng-Yan Liu Lu-Hao Li Zhao-Chen Liu Su-Xin Li Xiao-wei Dang Department of Hepatopancreatobiliary Surgery the First Affiliated Hospital of Zhengzhou UniversityZhengzhou 450052China Budd-Chiari Syndrome Diagnosis and Treatment Center of Henan Province Zhengzhou 450052China

Background:Hepatocellular carcinoma(HCC)is a serious complication of hepatic vena cava Budd-Chiari syndrome(HVC-BCS)that significantly reduces the survival time of patients.Our study aimed to analyze the prognostic factors influencing the survival of HVC-BCS patients with HCC and to develop a prognostic scoring system.Methods:The clinical and follow-up data of 64 HVC-BCS patients with HCC who received invasive treatment at the First Affiliated Hospital of Zhengzhou University between January 2015 and December 2019 were retrospectively analyzed.Kaplan-Meier curves and log-rank tests were used to analyze the survival curve of patients and the difference in prognoses between the groups.Univariate and multivariate Cox regression analyses were performed to analyze the influence of biochemical,tumor,and etiological characteristics on the total survival time of patients,and a new prognostic scoring system was developed according to the regression coefficients of the independent predictors in the statistical model.The prediction efficiency was evaluated using the time-dependent receiver operating characteristics curve and concordance index.Results:Multivariate analysis showed that serum albumin level7 cm(HR=8.623,95%CI:3.771-19.715,P<0.001),and inferior vena cava stenosis(HR=3.612,95%CI:1.646-7.928,P=0.001)were independent predictors of survival.A prognostic scoring system was developed according to the above-mentioned independent predictors,and patients were classified into grades A,B,C and D.Significant differences in survival were found among the four groups.Conclusions:This study successfully developed a prognostic scoring system for HVC-BCS patients with HCC,which is helpful for clinical evaluation of patient prognosis.

关键词： Budd-Chiari syndrome Hepatocellular carcinoma Prognostic factors Inferior vena cava stenosis Prognostic scoring system

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Chalcogen-dependent catalytic properties of RuX2 (X = S/Se/Te) nanoparticles decorated carbon nanofibers for hydrogen evolution in acidic and alkaline media

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Nano Research 2024年第4期17卷 2528-2537页

作者： Chengkai He Yue wei Jia Xu Yujie wei Tao Wang Rongfei Liu Lvlv Ji Zhun Liu sheng Wang School of Materials Science and Engineering Zhejiang Sci-Tech UniversityHangzhou 310018China Department of Physics Shaoxing UniversityShaoxing 312000China Zhejiang Kangjiesi New Material Technology Co. LTDZhuji 311800China

Transition metal dichalcogenides(TMDs),with the general formula MX_(2)(M=Mo/W/Fe/Co/Ni,etc.;X=S/Se/Te),have attracted extensive research interests for hydrogen evolution reaction(HER).Compared with numerous studies on noble-metal-free TMDs,the chalcogen-dependent HER catalytic properties of noble-metal-based TMDs are lack of sufficient research attention.Herein,a facile electrospinning-assisted synthetic strategy is proposed to synthesize ruthenium dichalcogenides(RuX_(2),X=S/Se/Te)nanoparticles decorated carbon nanofibers(CNFs).Benefiting from the identical nanofibrous morphology and exposed crystal planes of RuX_(2)(111),the catalytic activities of RuX_(2)@CNFs samples were investigated and compared in a fair and direct manner.Detailed electrochemical measurements coupled with density functional theory calculations were carried out to probe their intrinsic HER catalytic activities,resulting in the catalytic activity order of RuS_(2)@CNFs>RuSe_(2)@CNFs>RuTe_(2)@CNFs in acidic media and that of RuS_(2)@CNFs>RuTe_(2)@CNFs>RuSe_(2)@CNFs in alkaline media.The superior catalytic performance of RuS_(2)@CNFs mainly stems from the relative lower HER energy barriers and thereby the higher intrinsic catalytic activity of RuS_(2)(111),leading to ultralow overpotentials of 44 and 9 mV at 10 mA·cm^(-2) in acidic and alkaline media,respectively.RuSe_(2)(111)is endowed with the more optimized Gibbs free energy of hydrogen adsorption(ΔGH*)than RuTe_(2)(111),but RuTe_(2)(111)shows enhanced catalytic property for H_(2)O dissociation and OH-desorption than RuSe_(2)(111),therefore,resulting in the altered catalytic activity sequences for RuSe_(2) and RuTe_(2) in acidic and alkaline media.

关键词： hydrogen evolution reaction transition metal dichalcogenides carbon nanofibers electrocatalysis catalytic activity

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