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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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Targets capture by distributed active swarms via bio-inspired reinforcement learning

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Science China(Physics,Mechanics & Astronomy) 2025年第1期68卷 206-217页

作者： Kun Xu yue Li Jun sun Shuyuan Du Xinpeng Di Yuguang Yang Bo Li Institute of Biomechanics and Medical Engineering Applied Mechanics LaboratoryDepartment of Engineering MechanicsTsinghua UniversityBeijing 100084China Institute of Nuclear and New Energy Technology Tsinghua UniversityBeijing 100084China Shanghai Aerospace Control Technology Institute Shanghai 201109China Shanghai Key Laboratory of Aerospace Intelligent Control Technology Shanghai 201109China College of Automation Engineering Nanjing University of Aeronautics and AstronauticsNanjing 210016China

Natural swarms arranged from cells to herds are usually decentralized but display intriguing collective intelligence in coordinating individuals across large scales to efficiently achieve their common *** from nature may provide new strategies for controlling collective dynamics of synthetic swarms to accomplish specific ***,we present a bioinspired computational framework that steers distributed active swarms to collectively capture and merge targets via reinforcement *** exploit collective milling structures of natural herds to cage the targets,and adopt a switching control policy inspired by sperms’chiral dynamics to optimize the trajectories of individuals,through which the active swarms can selforganize to enclose single or multiple distant targets in a dynamical,adaptive and scalable *** exists a critical swarm size,beyond which the excessive competition between agents would generate large mechanical forces,leading to capture instability but enabling the transition from short-distance to long-distance merging capture of multiple *** work provides physical insights into distributed active swarms and could offer a multilevel,decentralized strategy toward controlling swarm robotics in wide applications such as bio-medical devices,machine immunity,and target clearance.

关键词： swarm intelligence robotics multi-tasks artificial intelligence bionics

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用旋转环盘电极测定n-砷化镓电极的稳定度

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太阳能学报 1989年第2期10卷 171-176页

作者：陆寿蕴孙月金承和复旦大学化学系

光生空穴与溶液中还原剂反应还是与半导体本身反应存在着竞争过程,本工作采用旋转环盘电极区分此竞争过程的两部分电流,研究了pH=1.0的硫酸亚铁和pH=9.2的亚铁氰化钾对n-砷化镓电极的稳定作用。结果表明,稳定度随还原剂浓度的增加、电... 详细信息

光生空穴与溶液中还原剂反应还是与半导体本身反应存在着竞争过程,本工作采用旋转环盘电极区分此竞争过程的两部分电流,研究了pH=1.0的硫酸亚铁和pH=9.2的亚铁氰化钾对n-砷化镓电极的稳定作用。结果表明,稳定度随还原剂浓度的增加、电流密度的降低而增加,但当溶液中pH增大时,砷化镓价带边的能级正移,在pH=9.2的亚铁氰化钾溶液中,价带边与亚铁氰化钾的已占领态重叠的机率很小,所以稳定度极小。结果还证明,稳定作用确是通过还原剂竞争光分解中间产物进行的,光分解为6电子的连续反应,还原剂竞争中间产物的结果,终止了分解反应,减少了光分解电流。

关键词：测定砷化镓稳定度电极

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Physics based circuit compatible model for hybrid antiferroelectric random access memory

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Science China(Information Sciences) 2025年第1期68卷 396-397页

作者： Qiuxia WU yue PENG Wenwu XIAO Wenxuan MA Shuo ZHANG Litao sun Chunfu ZHANG Xiaohua MA yue HAO School of Microelectronics Xidian University Xi'an Uni IC Semiconductors

Since the discovery of HfO2-based ferroelectric （FE） materials in 2011,considerable advancements have been made in material preparation,mechanism research,and device realization [1].Consequently,these materials are now regarded as leading candidates for embedded non-volatile memory（eNVM）.Beyond eNVM applications,recent studies have demonstrated that memory devices with antiferroelectric（AFE） films can achieve high response speeds,low latency,and reduced power consumption while maintaining excellent endurance characteristics [2,3].Additionally,the typical double hysteresis loops and multiple non-overlapping polarization current peaks of AFE films offer a novel approach to designing multistate ***,in order to further promote the application of high storage density AFE,an AFRAM electronic design automation （EDA） model must be developed,and it has yet to be proposed.

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A novel method for simultaneous measurement of^(222)Rn and^(220)Rn progeny concentrations measured by an alpha spectrometer

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Nuclear Science and Techniques 2025年第1期36卷 127-139页

作者： Zhong-Kai Fan Jia-Le sun Hao-Xuan Li Xiang-Ming Cai Hui Yang shou-Kang Qiu Yan-Liang Tan Jian Shan School of Nuclear Science and Technology University of South ChinaHengyang 421001China College of Physics and Electronic Engineering Hengyang Normal UniversityHengyang 421008China

The accumulation of^(222)Rn and^(220)Rn progeny in poorly ventilated environments poses the risk of natural radiation exposure to the public.A previous study indicated that satisfactory results in determining the^(222)Rn and^(220)Rn progeny concentrations by measuring the total alpha counts at five time intervals within 560 min should be expected only in the case of high progeny concentrations in *** complete the measurement within a relatively short period and adapt it for simultaneous measurements at comparatively lower^(222)Rn and^(220)Rn progeny concentrations,a novel mathematical model was proposed based on the radioactive decay *** model employs a nonlinear fitting method to distinguish nuclides with overlapping spectra by utilizing the alpha particle counts of non-overlapping spectra within consecutive measurement cycles to obtain the concentrations of^(222)Rn and^(220)Rn progeny in *** verification experiments were conducted using an alpha *** experimental results demonstrate that the concentrations of^(222)Rn and^(220)Rn progeny calculated by the new method align more closely with the actual circumstances than those calculated by the total count method,and their relative uncertainties are all within±16%.Furthermore,the measurement time was reduced to 90 min,representing an acceleration of 84%.The improved capability of the new method in distinguishing alpha particles with similar energies emitted from ^(218)Po and^(212)Bi,both approximately 6 MeV,contributed to realizing more accurate *** proposed method has the potential advantage of measuring relatively low concentrations of^(222)Rn and^(220)Rn progeny in air more quickly via air filtration.

关键词： ^(222)Rn ^(220)Rn Progeny concentration Nonlinear fitting method Alpha spectrometer

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Isothermal reduction kinetics and reduction prediction for iron ore pellets

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Journal of Iron and Steel Research International 2025年第1期32卷 64-72页

作者： Fei Meng Hao Liu yue-lin Qin Huang-jie Hua Yin Deng Ze-zheng sun Long-hai Liu School of Metallurgy and Materials Engineering Chongqing University of Science and TechnologyChongqing401331China CISDI Group Co. Ltd.Chongqing401122China College of Materials Science and Engineering Chongqing UniversityChongqing400044China Sichuan Desheng Group Vanadium&Titanium Co. Ltd.Leshan614000SichuanChina

Iron ore pellets,as one of the main charges of blast furnaces,have a greater impact on the CO_(2)emission reduction and stable operation of blast *** isothermal reduction behavior of the pellets obtained from a Chinese steel plant was studied in the gas mixtures of CO and N_(2).The results showed the reduction process is divided into two *** reduction in the initial stage(time t≤40 min)is cooperatively controlled by internal diffusion and interface chemical reactions with the activation energy of 30.19 and 16.67 kJ/mol,*** controlling step of the reduction in the final stage(t>40 min)is internal diffusion with the activation energy of 34.60 kJ/*** reduction process can be described by two equations obtained from kinetic *** reduction degree can be predicted under different temperatures and time,and the predicted results showed an excellent correlation with the experimental *** reduction mechanisms were confirmed by the analysis of the scanning electron microscope equipped with an energy dispersive spectrometer and optical microscope.

关键词： Pellet Reduction behavior Kinetics Mechanism Reduction prediction

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Assessment of future cotton production in the Tarim River Basin under climate model projections and water management

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Journal of Earth Science 2025年

作者： Shengru yue Lunche Wang Qian Cao Jia sun School of Geography and Information Engineering， China University of Geosciences College of Hydraulic and Architectural Engineering， Tarim University

Climate change is significantly impacting cotton production in the Tarim River Basin. The study investigated the climate change characteristics from 2021 to 2100 using climate change datasets simulated per the Coupled Model Inter-comparison Project phase six (CMIP6) climatic patterns under the Shared Socioeconomic Pathways SSP2-4.5 and SSP5-8.5. The DSSAT-CROPGRO-Cotton model， along with stepwise multiple regression analyses， was used to simulate changes in the potential yield of seed cotton due to climate change. The results show that while future temperatures in the Tarim River Basin will rise significantly， changes in precipitation and radiation during the cotton-growing season are minimal. Seed cotton yields are more sensitive to low temperatures than to precipitation and radiation. The potential yield of seed cotton under the SSP2-4.5 scenario would increase by 14.8%， 23.7%， 29.0%， and 29.4% in the 2030S， 2050S， 2070S， and 2090S， respectively. In contrast， under the SSP5-8.5 scenario， the potential yield of seed cotton would see increases of 17.5%， 27.1%， 30.1%， and 22.6% respectively. Except for the 2090s under the SSP5-8.5 scenario， future seed cotton production can withstand a 10% to 20% deficit in irrigation. These findings will help develop climate change adaptation strategies for cotton cultivation.

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Flexible artificial vision computing system based on FeOx optomemristor for speech recognition

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Journal of Semiconductors 2025年第1期46卷 225-232页

作者： Jie Li yue Xin Bai sun Dengshun Gu Changrong Liao Xiaofang Hu Lidan Wang Shukai Duan Guangdong Zhou College of Artificial Intelligence Southwest UniversityChongqing 400715China Frontier Institute of Science and Technology X’ian Jiaotong UniversityXi’an 710049China School of Electronic Information and Electrical Engineering Chongqing University of Arts and SciencesChongqing 402160China

With the advancement of artificial intelligence,optic in-sensing reservoir computing based on emerging semiconductor devices is high desirable for real-time analog signal ***,we disclose a flexible optomemristor based on C_(27)H_(30)O_(15)/FeOx heterostructure that presents a highly sensitive to the light stimuli and artificial optic synaptic features such as short-and long-term plasticity(STP and LTP),enabling the developed optomemristor to implement complex analogy signal processing through building a real-physical dynamic-based in-sensing reservoir computing algorithm and yielding an accuracy of 94.88%for speech *** charge trapping and detrapping mediated by the optic active layer of C_(27)H_(30)O_(15) that is extracted from the lotus flower is response for the positive photoconductance memory in the prepared *** work provides a feasible organic−inorganic heterostructure as well as an optic in-sensing vision computing for an advanced optic computing system in future complex signal processing.

关键词： reservoir computing flexible optomemristor analogy signal processing optic computing

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Characterization of petrophysical and seismic properties for CO2 storage with sensitivity analysis

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Petroleum Science 2025年第1期22卷 193-209页

作者： Yan-Jiao Dong Yi Shen Kai Guo Xiao-Qin Wu Qiang Mao Wen-yue sun Zhi-Qiang Wang State Key Laboratory of Offshore Oil and Gas Exploitation Geosciences Department China University of Petroleum (East China) CNOOC Research Institute Ltd. School of Petroleum Engineering China University of Petroleum (East China)

Saline aquifers are considered as highly favored reservoirs for CO2sequestration due to their favorable properties. Understanding the impact of saline aquifer properties on the migration and distribution of CO2plume is crucial. This study focuses on four key parameters—permeability, porosity, formation pressure, and temperature—to characterize the reservoir and analyse the petrophysical and elastic response of CO2. First, we performed reservoir simulations to simulate CO2saturation, using multiple sets of these four parameters to examine their significance on CO2saturation and the plume migration ***, the effect of these parameters on the elastic properties is tested using rock physics *** established a relationship of compressional wave velocity（Vp） and quality factor（Qp） with the four key parameters, and conducted a sensitivity analysis to test their sensitivity to Vpand Qp. Finally, we utilized visco-acoustic wave equation simulated time-lapse seismic data based on the computed Vpand Qpmodels, and analysed the impact of CO2saturation changes on seismic data. As for the above numerical simulations and analysis, we conducted sensitivity analysis using both homogeneous and heterogeneous models. Consistent results are found between homogeneous and heterogeneous models. The permeability is the most sensitive parameter to the CO2saturation, while porosity emerges as the primary factor affecting both Qpand Vp. Both Qpand Vpincrease with the porosity, which contradicts the observations in gas reservoirs. The seismic simulations highlight significant variations in the seismic response to different parameters. We provided analysis for these observations, which serves as a valuable reference for comprehensive CO2integrity analysis, time-lapse monitoring, injection planning and site selection.

关键词： CO2 storage Time-lapse seismic CO2 plume Sensitivity analysis Rock physics Reservoir simulation Saline aquifer

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Exhumation and Preservation of the Yangchuling Porphyry W-Mo Deposit in the Jiangnan Tungsten Ore Belt,South China:Insights from(U-Th)/He and Fission Track Dating

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Journal of Earth Science 2025年第1期36卷 46-56页

作者： yue sun Ying Wang Jianzhang Pang Samuel C.Boone Minting Wu Chongjian Shao Wei Liu Jiangxi Provincial Key Laboratory of Genesis and Prospect for Strategic Minerals School of Earth ScienceEast China University of TechnologyNanchang330013China Institute of Geology China Earthquake Administration Beijing100029China School of Geography Earth and Atmospheric Sciences The University of MelbourneMelbourneVIC3010Australia Department of Earth Sciences The University of AdelaideAdelaideSA5005Australia Jiangxi DuChang JinDing Tungsten CO. Ltd.Jiujiang332611China

The Yangchuling porphyry W-Mo deposit(YPWD),located in the Jiangnan porphyryskarn tungsten ore belt,is one of the most important and large-scale porphyry W-Mo deposits in South *** previous zircon U-Pb and molybdenite Re-Os data suggest that Yangchuling WMo ore bodies formed almost simultaneously with granodiorite and monzogranitic porphyry at~150–144 Ma,their post emplacement history remains poorly understood,making their preservation status at depth *** this paper,new zircon and apatite(U-Th)/He and apatite fission track(ZHe,AHe and AFT,respectively)data of one hornfels and five intrusive rocks from a 1000-meter borehole are ***,together with new inverse thermal history models and previous geochronological data,help elucidate the post-diagenetic exhumation history and preservation status of the Yangchuling porphyry W-Mo *** general,ZHe and AHe ages decrease gradually from the near surface downwards and have relatively little intra-sample variation,ranging from 133 to 73Ma and 67 to 25 Ma,*** four granodiorites yield similar AFT ages that range from 63 to 55 Ma with mean track lengths varying from 12.2±0.7 to 12.6±0.5μ*** history modelling indicates that the Yangchuling ore district experienced slow,monotonic cooling since the ***-depth relationships are interpreted as recording~3.7±0.8 km of Cretaceous-recent exhumation in response to regional extension throughout South China thought to have been driven by subduction retreat of the Paleo-Pacific *** of estimated net exhumation and previous metallogenic depth of~4–5 km suggests that W-Mo ore bodies could still exist at depths of up to~1.3±0.8 km relative to Earth surface in the YPWD *** of the YPWD is attributed to the limited amount of regional denudation during the Late Cretaceous and Cenozoic.

关键词： (U-Th)/He fission track ore preservation thermochronology Yangchuling porphyry W-Mo deposit

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