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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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Multi-scenario Simulation of the Impact of Land Use Change on the Ecosystem Service Value in the Suzhou-Wuxi-Changzhou Metropolitan Area,China

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Chinese Geographical Science 2024年第1期34卷 79-92页

作者： WANG Bin hu Chunguang zhang Yushuo Department of Tourism Management Soochow UniversitySuzhou215123China Academy of Culture and Tourism Research Soochow UniversitySuzhou215123China School of Architecture and Urban Planning Huazhong University of Science and TechnologyWuhan430074China hubei Engineering and Technology Research Center of Urbanization Wuhan430074China Faculty of Culture Tourism Shanxi University of Finance and EconomicsTaiyuan030006China

As the most economically developed metropolitan area in China’s Yangtze River Delta,the rapid changing land use patterns of Suzhou-Wuxi-Changzhou(Su-Xi-Chang) metropolitan area have profound impacts on the ecosystem service value(ESV).Based on the patterns of land use change and the ESV change in Su-Xi-Chang metropolitan area from 2000 to 2020,we set up four scenarios:natural development scenario,urban development scenario,arable land protection scenario and ecological protection scenario,and simulated the impact of land use changes on the ESV in these *** results showed that:1) the area of built-up land in the Su-XiChang metropolitan area increased significantly from 2000 to 2020,and the area of other types of land *** land underwent the highest transfer-out area,and was primarily converted into built-up *** total ESV of Su-Xi-Chang metropolitan area increased initially then declined from 2000–2020,and the value of almost all individual ecosystem services decreased.2) Population density,GDP per area,night lighting intensity,and road network density can negatively impact the ESV.3) The total ESV loss under the natural development and urban development scenarios was high,and the expansion of the built-up land and the drastic shrinkage of the arable land contributed to the ESV decline under both *** total ESV under arable land protection and ecological protection scenarios increases,and therefore these scenarios are suitable for future land use optimization in *** study could provide a certain reference for land use planning and allocation,and offer guidance for the rational allocation of land resources.

关键词： multiple scenarios land use simulation ecosystem service value(ESV) Patch-Generating Land Use Simulation(PLUS)model Suzhou-Wuxi-Changzhou(Su-Xi-Chang)metropolitan area

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Mechanical behaviors of warm and ice-rich frozen soil stabilized with sulphoaluminate cement

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Journal of Mountain Science 2024年第1期21卷 335-345页

作者： WANG Honglei zhang hu zhang Jianming zhang Qi YIN Zhenhua State Key Laboratory of Frozen Soil Engineering Northwest Institute of Eco-Environment and ResourcesChinese Academy of SciencesLanzhou 730000China University of Chinese Academy of Sciences Beijing 100049China School of Civil Engineering and Transportation Northeast Forestry UniversityHarbin 150040China National Key Laboratory of Green and Long-Life Road Engineering in Extreme Environment CCCC First Highway Consultants Co.LtdXi'an 710065China Railway Engineering Research Institute China Academy of Railway Sciences Corporation LimitedBeijing 100081China

The warm and ice-rich frozen soil is characterized by high unfrozen water content, low shear strength and large compressibility, which is unreliable to meet the stability requirements of engineering infrastructures and foundations in permafrost regions. In this study, a novel approach for stabilizing the warm and ice-rich frozen soil with sulphoaluminate cement was proposed based on chemical stabilization. The mechanical behaviors of the stabilized soil, such as strength and stress-strain relationship, were investigated through a series of triaxial compression tests conducted at -1.0℃, and the mechanism of strength variations of the stabilized soil was also explained based on scanning electron microscope test. The investigations indicated that the strength of stabilized soil to resist failure has been improved, and the linear Mohr-Coulomb criteria can accurately reflect the shear strength of stabilized soil under various applied confining pressure. The increase in both curing age and cement mixing ratio were favorable to the growth of cohesion and internal friction angle. More importantly, the strength improvement mechanism of the stabilized soil is attributed to the formation of structural skeleton and the generation of cementitious hydration products within itself. Therefore, the investigations conducted in this study provide valuable references for chemical stabilization of warm and ice-rich frozen ground, thereby providing a basis for in-situ ground improvement for reinforcing warm and ice-rich permafrost foundations by soil-cement column installation.

关键词： Permafrost regions Frozen soil Mechanical behavior Chemical stabilization Ground improvement Ground modification

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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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Optimization of microstructure design for enhanced sensing performance in flexible piezoresistive sensors

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Journal of Advanced Ceramics 2024年第6期13卷 711-728页

作者： Xinghao Fan Hailong hu Bin Liao Yaqi zhang Fan zhang School of Minerals Processing and Bioengineering Central South UniversityChangsha 410083China Research Institute of Aerospace Technology Central South UniversityChangsha 410083China

Flexible piezoresistive strain sensors have received significant attention due to their diverse applications in monitoring human activities and health,as well as in robotics,prosthetics,and human–computer interaction *** the various flexible sensor types,those with microstructure designs are considered promising for strain sensing due to their simple structure,high sensitivity,extensive operational range,rapid response time,and robust *** review provides a concise overview of recent advancements in flexible piezoresistive sensors based on microstructure design for enhanced strain sensing performance,including the impact of microstructure on sensing mechanisms,classification of microstructure designs,fabrication methods,and practical ***,this review delves into the analysis of piezoresistive sensor sensing mechanisms and performance parameters,exploring the relationship between microstructure design and performance ***,an in-depth discussion is presented,focusing on the primary themes of microstructure design classification,process selection,performance characteristics,and specific *** review employs mathematical modeling and hierarchical analysis to emphasize the directionality of different microstructures on performance enhancement and to highlight the performance advantages and applicable features of various microstructure *** conclusion,this review examines the multifunctionality of flexible piezoresistive sensors based on microstructure design and addresses the challenges that still need to be overcome and improved,such as achieving a wide range of stretchability,high sensitivity,and robust *** review summarizes the research directions for enhancing sensing performance through microstructure design,aiming to assist in the advancement of flexible piezoresistive sensors.

关键词： microstructure design sensing performance flexible piezoresistive strain sensors microsensing mechanism

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Glacial-interglacial cycles recorded in clay minerals from the eastern Tibetan Plateau over the last 800 ka and their indications

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Science China Earth Sciences 2024年第7期67卷 2162-2177页

作者： xiaoyan zhang Chunxia zhang Yan ZHAO Bin hu Zhengtang GUO Key Laboratory of Cenozoic Geology and Environment Institute of Geology and GeophysicsChinese Academy of SciencesBeijing 100029China College of Earth and Planetary Sciences University of Chinese Academy of SciencesBeijing 100049China College of Earth Science and Engineering Shandong University of Science and TechnologyQingdao 266590China Key Laboratory of Land Surface Pattern and Simulation Institute of Geographic Sciences and Natural Resources ResearchChinese Academy of SciencesBeijing 100101China

Clay mineral proxies have been widely used to reconstruct changes in provenance and the ***,high-altitude clay mineral indications and long-term and orbital variations have not been fully ***,we present an800-ka clay-mineral record from a lacustrine sediment core(ZB13-C2)from the Zoige Basin on the eastern Tibetan Plateau(TP).X-ray diffraction and scanning electron microscopy were used to identify the clay mineral assemblages and their micromorphological characteristics of core *** results revealed that the clay minerals in core ZB13-C2 are mainly illite,with small amounts of chlorite,kaolinite,and smectite(including irregular mixed-layers of illite-smectite(I/S)and chloritesmectite(C/S)).The clay mineral assemblages and proxies exhibit three stages of change,which are mainly controlled by the Zoige watershed range under different climate *** watershed was limited to the southeastern region with altitudes ranging from~3500 to~4200 meters above sea level(m a.s.l.),and glacial-interglacial hydraulic erosion controlled the input of bedrock debris during 800-621 ka *** expanded to higher altitudes(~4200 to~4700 m a.s.l.)due to stronger seasonal freezethaw weathering without significant glacial-interglacial variations under a cooling and drying climate during 621-100 ka ***,it expanded to its highest altitudes of~4700 to~5200 m *** of the watershed after 100 ka BP,and hydraulic erosion increased obviously under a warm climate of the interstadial MIS 3 on the *** watershed underwent two major expansions under different climatic conditions at~621 ka and 100 ka BP,with the wet/dry alternation became strong at~621 ka *** results reveal that long-term temperature changes and the wet/dry alternation at high altitudes controlled the watershed range and degree of the physical erosion,and that the indications of clay minerals should be carefully clarified before they are used as climate indicators,especi

关键词： Quaternary Zoige Basin Lacustrine sediments Paleoenvironment Weathering

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Multi-omics analysis of human tendon adhesion reveals that ACKR1-regulated macrophage migration is involved in regeneration

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Bone Research 2024年第2期12卷 390-406页

作者： Xinshu zhang Yao xiao Bo hu Yanhao Li Shaoyang zhang Jian Tian Shuo Wang Zaijin Tao Xinqi Zeng Ning-Ning Liu Baojie Li Shen Liu Department of Orthopaedics Shanghai Sixth People’s Hospital Affiliated to Shanghai Jiao Tong University School of Medicine600 Yishan RdShanghai 200233PR China Section of Spine Surgery Department of OrthopaedicsChangzheng HospitalNaval Medical UniversityShanghai 200003PR China Bio-X Institutes Key Laboratory for the Genetics of Developmental and Neuropsychiatric DisordersMinistry of EducationShanghai Jiao Tong UniversityShanghai 200241PR China Department of Orthopaedics Wuxi Ninth People’s Hospital Affiliated to Soochow UniversityWuxi 214062PR China State Key Laboratory of Systems Medicine for Cancer Center for Single-Cell OmicsSchool of Public HealthShanghai Jiao Tong University School of MedicineShanghai 200025China

Tendon adhesion is a common complication after tendon injury with the development of accumulated fibrotic tissues without effective anti-fibrotic therapies,resulting in severe *** are widely recognized as a fibrotic trigger during peritendinous adhesion ***,different clusters of macrophages have various functions and receive multiple regulation,which are both still *** our current study,multi-omics analysis including single-cell RNA sequencing and proteomics was performed on both human and mouse tendon adhesion tissue at different stages after tendon *** transcriptomes of over 74000 human single cells were *** results,we found that SPP1^(+)macrophages,RGCC^(+)endothelial cells,ACKR1^(+)endothelial cells and ADAM12^(+)fibroblasts participated in tendon adhesion ***,despite specific fibrotic clusters in tendon adhesion,FOLR2^(+)macrophages were identified as an antifibrotic cluster by in vitro experiments using human ***,ACKR1 was verified to regulate FOLR2^(+)macrophages migration at the injured peritendinous site by transplantation of bone marrow from Lysm-Cre;R26R^(tdTomato) mice to lethally irradiated Ackr1^(-/-)mice(Ackr1^(-/-)chimeras;deficient in ACKR1)and control mice(WT chimeras).Compared with WT chimeras,the decline of FOLR2^(+)macrophages was also observed,indicating that ACKR1 was specifically involved in FOLR2^(+)macrophages *** together,our study not only characterized the fibrosis microenvironment landscape of tendon adhesion by multi-omics analysis,but also uncovered a novel antifibrotic cluster of macrophages and their *** results provide potential therapeutic targets against human tendon adhesion.

关键词： tendon analysis landscape

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Effect of samarium doping on the activity and sulfur resistance of Ce/MnFeO_(x) catalyst for low-temperature selective catalytic reduction of NO_(x) by ammonia

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Chinese Journal of Chemical Engineering 2024年第7期71卷 272-282页

作者： Qiyao zhang Shuangshuang zhang Xu hu Yongmin huang Key Laboratory of Specially Functional Polymeric Materials and Related Technology School of Chemistry and Molecular EngineeringEast China University of Science and TechnologyShanghai 200237China

The self-made MnFeO catalysts doped with cerium and samarium were prepared by impregnation method for low-temperature selective catalytic reduction(SCR)by *** this work,the surface properties of the series of MnFe-based catalysts were *** results indicate Sm-modified catalyst have superior low-temperature SCR activity;NO_(x) conversion maintained at nearby to 100%at 90℃ to 240℃.In addition,The N2 selectivity of Sm doping remains above 80%in the range of 60℃ to 150℃.In SO_(2) poisoning test,the NO_(x) conversion can be remained>90%after 10 h of *** XPS,NH3-TPD and H_(2)-TPR results show the catalyst with Sm doping enhances the acid sites and oxidation catalytic sites of mixed oxides serves for improving oxygen vacancies and transfer *** situ diffuse reflaxions infrared Fourier transformations spectroscopy(DRIFTS)results show that NO_(x) is more easily adsorbed on the surface after Sm doping,which provided favorable conditions for the NH3-SCR reaction to *** reaction at the catalyst surface will follow the L-H reaction mechanism by transient reaction test.

关键词： SCR Sm doping Catalyst Sulfur resistance In situ DRIFTS Selectivity

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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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Recent advances in electrochemical performance of Mg-based electrochemical energy storage materials in supercapacitors:Enhancement and mechanism

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Journal of Magnesium and Alloys 2024年第1期12卷 35-58页

作者： Yuntao xiao Xinfang zhang Can Wang Jinsong Rao Yuxin zhang College of Materials Science and Engineering Chongqing UniversityChongqing 400044China

The application of Mg-based electrochemical energy storage materials in high performance supercapacitors is an essential step to promote the exploitation and utilization of magnesium resources in the field of energy ***,the inherent chemical properties of magnesium lead to poor cycling stability and electrochemical reactivity,which seriously limit the application of Mg-based materials in ***,in this review,more than 70 research papers published in recent 10 years were collected and *** representative research works were selected,and the results of various regulative strategies to improve the electrochemical performance of Mg-based materials were *** effects of various regulative strategies(such as constructing nanostructures,synthesizing composites,defect engineering,and binder-free synthesis,etc.)on the electrochemical performance and their mechanism are demonstrated using spinelstructured MgX_(2)O_(4) and layered structured Mg-X-LDHs as *** addition,the application of magnesium oxide and magnesium hydroxide in electrode materials,MXene's solid spacers and hard templates are ***,the challenges and outlooks of Mg-based electrochemical energy storage materials in high performance supercapacitors are also discussed.

关键词： Supercapacitor Electrochemical energy storage Mg-based materials

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