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Clinical and molecular characterization of 10 Chinese children with congenital adrenal hyperplasia due to 11beta‑hydroxylase deficiency

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World Journal of Pediatrics 2024年第4期20卷 422-433页

作者： Wen‑Li Lu Xiao‑Yu Ma Jiao Zhang Jun‑Qi Wang Ting‑Ting Zhang Lei Ye Yuan Xiao Zhi‑Ya Dong Wei Wang shou‑yue sun Chuan‑Yin Li Rong‑Gui Hu Guang Ning Li‑Dan Zhang Department of Pediatrics Ruijin HospitalShanghai Jiao Tong University School of MedicineNo.197Ruijin 2nd RdHuangpu DistrictShanghai 200025China Department of Pediatric Genetic and Metabolic Endocrinology West China Second University HospitalSichuan UniversityNo.20Section 3Renmin South RoadSichuan 610041China Department of Endocrine and Metabolic Diseases Shanghai Clinical Center for Endocrine and Metabolic DiseasesRuijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Cancer Center School of MedicineShanghai Tenth People’s HospitalTongji UniversityYanchang Zhong Lu 301St RdJing’an DistrictShanghai 200031China

Background The clinical manifestations of nonclassical 11beta-hydroxylase deficiency are very similar to those of nonclassical 21-hydroxylase *** this study,we investigated the relationship between the clinical and molecular features of congenital adrenal hyperplasia caused by 11beta-hydroxylase deficiency and reviewed the related literature,which are expected to provide assistance for the clinical diagnosis and analysis of congenital adrenal *** Clinical data for 10 Chinese patients diagnosed with congenital adrenal hyperplasia in our hospital from 2018 to 2022 were retrospectively *** examined the effects of gene mutations on protease activity and constructed threedimensional structure prediction models of *** We describe 10 patients with 11beta-hydroxylase gene mutations(n=5,46,XY;n=5,46,XX),with 10 novel mutations were *** patients received treatment at an early stage,with an average age of 2.08±1.66 years,whereas male patients received treatment significantly later,at an average age of 9.77±3.62 *** most common CYP11B1 pathogenic variant in the Chinese population was found to be c.1360C>*** mutations lead to spatial conformational changes that affect protein *** Our study found that there was no significant correlation between each specific mutation and the severity of clinical *** patients with the same gene pathogenic variant may have mild or severe clinical *** correlation between genotype and phenotype needs further ***-dimensional protein simulations may provide additional support for the physiopathological mechanism of genetic mutations.

关键词： 11β-hydroxylase deficiency CYP11B1 Enzymatic activity Phenotype-genotype correlation Threedimensional protein simulations

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Optimizing evasive maneuvering of planes using a flight quality driven model

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Science China(Information Sciences) 2024年第3期67卷 197-208页

作者： Chang LIU Shaoshan sun Chenggang TAO Yingxin shou Bin XU School of Automation Northwestern Polytechnical University Chengdu Aircraft Design & Research Institute of AVIC

This paper investigates the optimal evasive maneuver for a plane to avoid an incoming missile. To accurately model the system dynamics while improving computational efficiency, a simplified plane model based on flight quality is established. A missile model with proportional guidance is also *** problem of determining the optimal evasion plane maneuver is formulated. The Gauss pseudospectral method(GPM) is proposed as a solution to find the optimal maneuver. The optimized maneuver is validated by testing it on a high-fidelity 6-degree-of-freedom(6-DOF) model, which demonstrates the effectiveness of the proposed simplified plane model. The Monte Carlo method is employed to evaluate the capability of the plane to evade missiles in various scenarios and elucidate general principles for successful evasion.

关键词： optimal maneuver flying quality evasive strategy pursuit-evasion game air-to-air combat

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On the global smooth solutions of 3D incompressible Hall-MHD equations

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Applied Mathematics(A Journal of Chinese Universities) 2024年第2期39卷 319-327页

作者： yue Gao-cheng sun Wen-da School of Mathematics Nanjing University of Aeronautics and AstronauticsNanjing 211106China

The present paper is devoted to the well-posedness issue for the 3D incompressible Hall-MHD system obtained from kinetic models. Our analysis strongly relies on the use of the Fourier analysis. We establish the global existence of smooth solutions for a class of large initial data, this result implies the initial velocity and magnetic field can be arbitrarily large.

关键词： 3D Hall-MHD well-posedness Fourier analysis

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Cascade electrolysis and thermocatalysis: a reliable system for upgrading C1 to C4 hydrocarbons

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Rare Metals 2024年第1期43卷 410-412页

作者： Chao-yue sun Wen Li Hai-Qing Wang Institute for Advanced Interdisciplinary Research(iAIR) College of Chemistry and Chemical EngineeringUniversity of JinanJinan 250022China

The carbon cycle is important for maintaining a stable climate and carbon balance on Earth. Renewable-electricitydriven upcycling of carbon dioxide (CO_(2)) into value-added multi-carbon molecules is a potentially sus... 详细信息

关键词： cycling hydrocarbons enable

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Natural marmatite photocatalyst for treatment of mineral processing wastewater to help zero wastewater discharge

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Journal of Environmental Sciences 2024年第8期142卷 83-91页

作者： Meirong Wu Wei sun Xiangsong Meng Jianhua Kang yue Yang School of Minerals Processing and Bioengineering Central South UniversityChangsha 410083China Key Laboratory of Hunan Province for Clean and Efficient Utilization of Strategic Calcium-containing Mineral Resources Central South UniversityChangsha 410083China

Mineral processing wastewater(MPW)with large discharge and high toxicity affects environmental safety,and the realizing zero discharge of MPW is of great significance for reducing environmental pollution,saving water resources,and promoting the sustainable development of the mining *** this study,we reported natural marmatite(NM)as a low-cost and efficient photocatalyst for the treatment of MPW to help zero wastewater *** photocatalytic activity of NM was evaluated by the removal of total organic carbon(TOC)from MPW under visible-light illumination,and the optimal degradation conditions were *** showed that superoxide free radicals(^(·)O_(2)^(-))were the dominant active species responsible for organic pollutants degradation,and 74.25%TOC removal was obtained after 120 min reaction under the optimum treatment ***,the wastewater treated by NM photocatalysis can be reused in the flotation system without adverse impact on the product *** on these findings,a model of zero wastewater discharge for flotation with the help of photocatalytic treatment was established,it indicated that the water of the whole system can be balanced without affecting the ore dressing index,which showed that visible light-driven photocatalyst has a promising application prospect in the treatment and recycling of industrial wastewater.

关键词： Natural mineral Photocatalysis Industrial wastewater Wastewater recycling Water balance

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Full-domain collaborative deployment method of multiple interference sources and evaluation of its deployment effect

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Defence Technology（防务技术） 2024年第2期32卷 572-595页

作者： yue Wang Fuping sun Xian Wang Jinming Hao Kai Xiao School of Geospatial Information The PLA Information Engineering UniversityZhengzhou 450001HenanChina

This paper realizes the full-domain collaborative deployment of multiple interference sources of the global satellite navigation system(GNSS)and evaluates the deployment effect to enhance the ability to disturb the attacker and the capability to defend the GNSS during navigation *** evaluation indicators for the jamming effect of GNSS suppressive and deceptive jamming sources are first created,their evaluation models are built,and their detection procedures are sorted out,as the basis for determining the deployment *** principles for collaboratively deploying multi-jamming sources are developed to obtain the deployment structures(including the required number,structures in demand,and corresponding positions)of three single interference sources required by collaboratively ***,simulation and hardware-in-loop testing results are presented to determine a rational configuration of the collaborative deployment of multi-jamming sources in the set situation and further realize the full-domain deployment of an interference network from ground,air to *** evaluation indices for the deployment effect are finally developed to evaluate the deployment effect of the proposed configuration and further verify its reliability and rationality.

关键词： Jamming effect Multiple interference sources Collaborative deployment Effect evaluation Defense capability

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Smart Gait:A Gait Optimization Framework for Hexapod Robots

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Chinese Journal of Mechanical Engineering 2024年第1期37卷 146-159页

作者： Yunpeng Yin Feng Gao Qiao sun yue Zhao Yuguang Xiao State Key Laboratory of Mechanical System and Vibration School of Mechanical EngineeringShanghai Jiao Tong UniversityShanghai 200240China Lenovo Corporation ShanghaiChina AI Institute School of Electronic Information and Electrical EngineeringShanghai Jiao Tong UniversityShanghai 200240China

The current gait planning for legged robots is mostly based on human presets,which cannot match the flexible characteristics of natural *** paper proposes a gait optimization framework for hexapod robots called Smart *** Gait contains three modules:swing leg trajectory optimization,gait period&duty optimization,and gait sequence *** full dynamics of a single leg,and the centroid dynamics of the overall robot are considered in the respective *** Smart Gait not only helps the robot to decrease the energy consumption when in locomotion,mostly,it enables the hexapod robot to determine its gait pattern transitions based on its current state,instead of repeating the formalistic clock-set step *** Smart Gait framework allows the hexapod robot to behave nimbly as a living animal when in 3D movements for the first *** Smart Gait framework combines offline and online optimizations without any fussy data-driven training procedures,and it can run efficiently on board in real-time after *** experiments are carried out on the hexapod robot *** results show that the energy consumption is reduced by 15.9%when in *** gait patterns can be generated spontaneously both in regular and challenge environments,and when facing external interferences.

关键词： Gait optimization Swing trajectory optimization Legged robot Hexapod robot

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Paleosalinity reconstruction in offshore lacustrine basins based on elemental geochemistry: a case study of Middle-Upper Eocene Shahejie Formation, Zhanhua Sag, Bohai Bay Basin

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JOURNAL OF OCEANOLOGY AND LIMNOLOGY 2024年第4期42卷 1-19页

作者： sun, Long Wu, Shenghe yue, Dali Cui, Wenfu China Univ Petr Natl Key Lab Petr Resources & Engn Beijing 102249 Peoples R China China Univ Petr Coll Geosci Beijing 102249 Peoples R China SINOPEC Shengli Oilfield Co Shengli Oil Prod Plant Dongying 257400 Peoples R China

Salinity is a crucial property of water body and is essential for the restoration of paleoecology and paleoenvironment. However, the theoretical method of application of elemental geochemical proxies to paleosalinity reconstruction is still underdeveloped. Moreover, accurate determination and reconstruction of paleosalinity and its variation in an offshore lacustrine basin have been extremely challenging thus far. This study presents detailed elemental geochemical investigations from the Zhanhua Sag in the southern Bohai Bay Basin to reconstruct the salinity variation in the Paleogene Eocene Shahejie Formation (50.8-33.9 Ma). Based on the variation of strontium barium ratio (Sr/Ba) and boron gallium ratio (B/Ga), we determined three typical salinity types of water body: salty water (Sr/Ba>0.5, B/Ga>6), brackish water (0.2

关键词： paleosalinity paleoclimate Zhanhua Sag Middle-Upper Eocene Shahejie Formation EASTERN CHINA YANGTZE DELTA RED BEDS DEPRESSION MARINE BORON STRATIGRAPHY EVOLUTION MUDSTONE RECORD

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Recent advances in stretchable triboelectric nanogenerators for use in wearable bioelectronic devices

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Bio-Design and Manufacturing 2024年第4期7卷 566-590页

作者： Yaling Wang Pengcheng Zhu yue sun Pan Li Yanchao Mao College of Science Henan Agricultural UniversityZhengzhou 450002China Key Laboratory of Materials Physics of Ministry of Education School of PhysicsZhengzhou UniversityZhengzhou 450001China

Wearable bioelectronic devices have the capacity for real-time human health monitoring,the provision of tailored services,and natural interaction with smart ***,these wearable bioelectronic devices rely on conventional rigid batteries that are frequently charged or replaced and are incompatible with the skin,leading to a discontinuity in complex therapeutic tasks related to human health monitoring and human-machine *** triboelectric nanogenerator(TENG)is a high-efficiency energy harvesting technology that converts mechanical into electrical energy,effectively powering wearable bioelectronic *** study comprehensively overviews recent advances in stretchable TENG for use in wearable bioelectronic *** working mechanism of stretchable TENG is initially explained.A comprehensive discussion presents the approaches for fabricating stretchable TENG,including the design of stretchable structures and the selection of stretchable ***,applications of wearable bioelectronic devices based on stretchable TENG in human health monitoring(body movements,pulse,and respiration)and human-machine interaction(touch panels,machine control,and virtual reality)are ***,the challenges and developmental trends regarding wearable bioelectronic devices based on stretchable TENG are elaborated.

关键词： Stretchable Triboelectric nanogenerators Structure Human health monitoring Human-machine interaction

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Data-driven casting defect prediction model for sand casting based on random forest classification algorithm

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China Foundry 2024年第2期21卷 137-146页

作者： Bang Guan Dong-hong Wang Da Shu shou-qin Zhu Xiao-yuan Ji Bao-de sun Shanghai Key Lab of Advanced High-Temperature Materials and Precision Forming School of Materials Science and EngineeringShanghai Jiao Tong UniversityShanghai 200240China State Key Lab of Metal Matrix Composites Shanghai Jiao Tong UniversityShanghai 200240China Hefei Casting and Forging Factory of Anhui Heli Co. LtdHefei 230000China State Key Laboratory of Materials Processing and Die&Mould Technology Huazhong University of Science and TechnologyWuhan 430074China

The complex sand-casting process combined with the interactions between process parameters makes it difficult to control the casting quality,resulting in a high scrap rate.A strategy based on a data-driven model was proposed to reduce casting defects and improve production efficiency,which includes the random forest(RF)classification model,the feature importance analysis,and the process parameters optimization with Monte Carlo *** collected data includes four types of defects and corresponding process parameters were used to construct the RF *** results show a recall rate above 90% for all *** Gini Index was used to assess the importance of the process parameters in the formation of various defects in the RF ***,the classification model was applied to different production conditions for quality *** the case of process parameters optimization for gas porosity defects,this model serves as an experimental process in the Monte Carlo method to estimate a better temperature *** prediction model,when applied to the factory,greatly improved the efficiency of defect *** show that the scrap rate decreased from 10.16% to 6.68%.

关键词： sand casting process data-driven method classification model quality prediction feature importance

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