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Clinical manifestations and spermatogenesis outcomes in Chinese patients with congenital hypogonadotropic hypogonadism caused by inherited or de novo FGFR1 mutations

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Asian Journal of Andrology 2024年第4期26卷 426-432页

作者： Yu-Fan yang Hai-Lu Ma Xi Wang Min Nie Jiang-feng Mao Xue-yan wu Department of Endocrinology Peking Union Medical College HospitalChinese Academy of Medical SciencesPeking Union Medical CollegeBeijing 100730China.

Fibroblast growth factor receptor 1(FGFR1)mutations are associated with congenital hypogonadotropic hypogonadism(CHH)through inheritance or spontaneous *** detected FGFR1 mutations in a Chinese cohort of 210 CHH patients at Peking Union Medical College Hospital(Beijing,China)using next-generation and Sanger *** assessed missense variant pathogenicity using six bioinformatics tools and compared clinical features and treatment outcomes between inherited and de novo mutation *** 19 patients with FGFR1 mutations,three were recurrent,and 16 were novel *** of the novel mutations were likely pathogenic according to the American College of Medical Genetics and Genomics(ACMG)guidelines,with the prevalent P366L *** majority of FGFR1 mutations was inherited(57.9%),with frameshift mutations exclusive to the de novo mutation *** inherited mutation group had a lower incidence of cryptorchidism,short stature,and skeletal *** the inherited mutation group,luteinizing hormone(LH)levels were 0.5 IU l−1,follicle-stimulating hormone(FSH)levels were 1.0 IU l−1,and testosterone levels were 1.3 nmol l−*** contrast,the de novo group had LH levels of 0.2 IU l−1,FSH levels of 0.5 IU l−1,and testosterone levels of 0.9 nmol l−1,indicating milder hypothalamus–pituitary–gonadal axis(HPGA)functional deficiency in the inherited *** inherited mutation group showed a tendency toward higher spermatogenesis *** conclusion,this study underscores the predominance of inherited FGFR1 mutations and their association with milder HPGA dysfunction compared to de novo mutations,contributing to our understanding of the genetic and clinical aspects of FGFR1 mutations.

关键词： congenital hypogonadotropic hypogonadism de novo FGFR1 inherited

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Performance improvements in complementary metal oxide semiconductor devices and circuits based on fin field-effect transistors using 3-nm ferroelectric Hf_(0.5)Zr_(0.5)O_(2)

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Rare Metals 2024年第7期43卷 3242-3249页

作者： Zhao-Hao Zhang yan-Na Luo Gao-Bo Xu Jia-Xin Yao Zhen-hua wu Hong-Bin Zhao Qing-Zhu Zhang hua-Xiang Yin Jun Luo Wen-wu Wang Hai-Ling Tu Integrated Circuit Advanced R&D Center Institute of Microelectronics of Chinese Academy of SciencesBeijing 100029China School of Integrated Circuits University of Chinese Academy of SciencesBeijing 100049China State Key Laboratory of Advanced Materials for Smart Sensing General Research Institute for Nonferrous MetalsBeijing 100088China

In this work,a conventional HfO_(2) gate dielectric layer is replaced with a 3-nm ferroelectric(Fe) HZO layer in the gate stacks of advanced fin field-effect transistors(FinFETs).Fe-induced characteristics,e.g.,negative drain induced barrier lowering(N-DIBL) and negative differential resistance(NDR),are clearly observed for both p-and n-type HZO-based *** characteristics are attributed to the enhanced ferroelectricity of the 3-nm hafnium zirconium oxide(HZO) film,caused by Al doping from the TiAlC capping *** mechanism is verified for capacitors with structures similar to the *** to the enhanced ferroelectricity and N-DIBL phenomenon,the drain current(I_(DS))of the HZO-FinFETs is greater than that of HfO_(2)-FinFETs and obtained at a lower operating ***,circuits based on HZO-FinFET achieve higher performance than those based on HfO_(2)-FinFET at a low voltage drain(V_(DD)),which indicates the application feasibility of the HZO-FinFETs in the ultralow power integrated circuits.

关键词： FinFET Ferroelectric Hafnium zirconium oxide Subthreshold swing Low power

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Quantitative characterizations of anisotropic dynamic properties in organic-rich shale with different kerogen content

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Petroleum Science 2024年第2期21卷 855-865页

作者： Jian-Yong Xie yan-Ping Fang Xing-hua wu Jian'er Zhao Jun-Cheng Dai Jun-Xing Cao Ji-Xin Deng State Key Laboratory of Oil and Gas Reservoir Geology and Exploitation Chengdu University of TechnologyChengdu610059SichuanChina Key Lab of Earth Exploration&Information Techniques of Ministry of Education Geophysical InstituteChengdu University of TechnologyChengdu610059SichuanChina PetroChina Southwest Oil&Gasfield Company Chengdu610017SichuanChina

Understanding the quantitative responses of anisotropic dynamic properties in organic-rich shale with different kerogen content(KC)is of great significance in hydrocarbon exploration and *** controlled experiments with a single variable is challenging for natural shales due to their high variations in components,diagenesis conditions,or pore *** employed the hot-pressing technique to construct 11 well-controlled artificial shale with varying *** artificial shale samples were successive machined into prismatic shape for ultrasonic measurements along different *** revealed bedding perpendicular P-wave velocities are more sensitive to the increasing KC than bedding paralleling velocities due to the preferential alignments of *** elastic stiffnesses except C_(13)are generally decreasing with the increasing KC,the variation of C_(1) and C_(33)on kerogen content are more sensitive than those of C_(44)and C_(66).Apparent dynamic mechanical parameters(v and E)were found to have linear correlation with the true ones from complete anisotropic equations independent of KC,which hold value towards the interpretation of well logs consistently across formations,Anisotropic mechanical parameters(ΔE and brittlenessΔB)tend to decrease with the reducing KC,withΔB showing great sensitivity to KC *** the range of low KC(10%)shale,compilation of data from natural organic rich-shales globally verified the similar systematic relationships that can be empirically used to predict the fraction of KC in shales.

关键词： Elastic properties Organic shale Anisotropy Kerogen content Physical modeling

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Effects of interstitial cluster mobility on dislocation loops evolution under irradiation of austenitic steel

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Nuclear Science and Techniques 2024年第8期35卷 69-78页

作者： Xin‑hua yan Lu Sun Du Zhou Teng Xie Chang Peng Ye‑Xin yang Li Chen Zhen‑feng Tong North China Electric Power University Beijing 102206China China Nuclear Power Operations Co. LtdShenzhen 518000China

The evolution of dislocation loops in austenitic steels irradiated with Fe^(+)is investigated using cluster dynamics(CD)simulations by developing a CD *** CD predictions are compared with experimental results in the *** number density and average diameter of the dislocation loops obtained from the CD simulations are in good agreement with the experimental data obtained from transmission electron microscopy(TEM)observations of Fe~+-irradiated Solution Annealed 304,Cold Worked 316,and HR3 austenitic steels in the *** CD simulation results demonstrate that the diffusion of in-cascade interstitial clusters plays a major role in the dislocation loop density and dislocation loop growth;in particular,for the HR3 austenitic steel,the CD model has verified the effect of temperature on the density and size of the dislocation loops.

关键词： Cluster dynamics Dislocation loops In-cascade interstitial clusters Austenitic steels

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Self-supported NiFe-LDH nanosheets on NiMo-based nanorods as high-performance bifunctional electrocatalysts for overall water splitting at industrial-level current densities

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Nano Research 2024年第5期17卷 3769-3776页

作者： yan Zhang Biao feng MingLei yan Zhen Shen Yiqun Chen Jingyi Tian fengfei Xu Guanghai Chen Xizhang Wang Lijun yang Qiang wu Zheng Hu Key Laboratory of Mesoscopic Chemistry of Ministry of Education(MOE)and Jiangsu Provincial Lab for Nanotechnology School of Chemistry and Chemical EngineeringNanjing UniversityNanjing 210023China College of Water Conservancy and Hydropower Engineering Sichuan Agricultural UniversityYa’an 625014China

Efficient,durable and economic electrocatalysts are crucial for commercializing water electrolysis ***,we report an advanced bifunctional electrocatalyst for alkaline water splitting by growing NiFe-layered double hydroxide(NiFe-LDH)nanosheet arrays on the conductive NiMo-based nanorods deposited on Ni foam to form a three-dimensional(3D)architecture,which exhibits exceptional performances for both hydrogen evolution reaction(HER)and oxygen evolution reaction(OER).In overall water splitting,only the low operation voltages of 1.45/1.61 V are required to reach the current density of 10/500 mA·cm^(-2),and the continuous water splitting at an industrial-level current density of 500 mA·cm^(-2) shows a negligible degradation(1.8%)of the cell voltage over 1000 *** outstanding performance is ascribed to the synergism of the HER-active NiMo-based nanorods and the OER-active NiFe-LDH nanosheet arrays of the hybridized 3D ***,the dense NiFe-LDH nanosheet arrays enhance the local pH on cathode by retarding OH-diffusion and enlarge the electrochemically active surface area on anode,while the conductive NiMo-based nanorods on Ni foam much decrease the charge-transfer resistances of both *** study provides an efficient strategy to explore advanced bifunctional electrocatalysts for overall water splitting by rationally hybridizing HER-and OER-active components.

关键词： alkaline water splitting bifunctional electrocatalysts layered double hydroxides high durability industrial current densities

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Flexible MXene/sodium alginate composite fabric with high structural stability and oxidation resistance for electromagnetic interference shielding

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Nano Research 2024年第6期17卷 5326-5335页

作者： Hao-Wen Zhang Lu-Yao yang Meng-Lin huang Ming-hua Cheng Zhe-Sheng feng Fanbin Meng Zifeng Lin yan Wang School of Materials and Energy University of Electronic Science and Technology of ChinaChengdu 610054China Laboratory of Advanced Technologies of Materials(Ministry of Education) School of Materials Science and EngineeringSouthwest Jiaotong UniversityChengdu 610031China College of Materials Science and Engineering Sichuan UniversityChengdu 610065China

Wearable electromagnetic interference(EMI)shielding fabrics with excellent electromagnetic shielding performance,oxidation resistance,and structural stability are highly demanded for the rapid development of electronic devices and wireless *** are metallic conductive materials with exceptional EMI shielding properties,but they are prone to oxidation in air and have poor structural stability and durability on fabric ***,we present a one-step assembly method to fabricate fabrics coated with MXenes and polymeric sodium alginate(SA)composite(MXene-SA).SA protects MXenes from oxidation and forms a stable interlayer structure by bonding to *** MXene-SA coated fabrics are breathable and flexible,and have a low sheet resistance of 2.12±0.08Ω/sq and a high EMI shielding performance of 37.05 dB at X-band,which is comparable to the best 42.31 ***,the MXene-SA coated fabrics exhibit high structural stability and oxidation resistance under various conditions of sonication disintegration,mechanical abuse,chemical corrosion,and humidity,compared to pure MXenes coated *** believe that the wearable and high-performance MXene-SA fabrics have great potential for the next generation of ultra-portable and wearable EMI shielding products.

关键词： MXenes electromagnetic interference shielding fabric one-step assembly

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Left ventricular thrombosis caused cerebral embolism during venoarterial extracorporeal membrane oxygenation support: A case report

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World Journal of Clinical Cases 2024年第5期12卷 973-979页

作者： Yao-Bang Bai feng Zhao Zhen-hua wu Guo-Ning Shi Nan Jiang Intensive Care Unit Department of Cardiac SurgeryTianjin Chest HospitalTianjin 300222China Department of Cardiac Surgery Tianjin Chest HospitalTianjin 300222China

BACKGROUND Venoarterial(VA)extracorporeal membrane oxygenation(ECMO),an effective short-term circulatory support method for refractory cardiogenic shock,is widely ***,retrospective analyses have shown that VA-ECMO-assisted cases were associated with a relatively high mortality rate of approximately 60%.Embolization in important organs caused by complications of left ventricular thrombosis(LVT)during VA-ECMO is also an important *** the incidence of LVT during VA-ECMO is not high,the consequences of embolization are *** SUMMARY A 37-year-old female patient was admitted to hospital because of fever for 4 d and palpitations for 3 *** excluding the diagnosis of coronary heart disease,we established a diagnosis of“clinically explosive myocarditis”.The patient still had unstable hemodynamics after drug treatment supported by VA-ECMO,with heparin for *** day 4 of ECMO support,a left ventricular thro-mbus attached to the papillary muscle root of the mitral valve was found by transthoracic *** ventricular decompression was performed and ECMO was successfully removed,but the patient eventually died of multiple cerebral *** LVT with high mobility during VA-ECMO may cause embolism in important ***,a"wait and see"strategy should be avoided.

关键词： Venoarterial extracorporeal membrane oxygenation Left ventricular thrombosis Cerebral embolism Magnetic Resonance Imaging Therapy Case report

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Enhanced activity and durability of FeCoCrMoCBY nanoglass in acidic hydrogen evolution reaction

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Journal of Materials Science & Technology 2024年第3期170卷 212-220页

作者： Mengyang yan Shuangqin Chen Shangshu wu Xuechun Zhou Shu Fu Di Wang Christian Kübel Horst Hahn Si Lan Tao feng Herbert Gleiter Institute of Nanoscience School of Material Science and EngineeringNanjing University of Science and TechnologyNanjing 210094China Institute of Nanotechnology Karlsruhe Institute of TechnologyKarlsruhe 76021Germany

In the present work,a multi-element nanoglass(m-NG)of FeCoCrMoCBY is obtained first time by the laser ablation combined with inert gas condensation(laser-IGC)*** with the conventional rapid-quenched metallic glass(MG)with identical composition,the Fe-based m-NG demonstrates a superior performance as a self-supported electrocatalyst for hydrogen evolution reaction(HER)in acidic *** enhanced HER activity of m-NG is proposed to be closely related to its high en-ergy states,which is originated from the unique inhomogeneous nanostructures with a high density of low-coordinated ***,the Fe-based m-NG exhibits an outstanding comprehensive catalytic performance even beyond the commercial Pt/C catalyst in long-term test due to its self-optimization *** work not only opens the way to the preparation of m-NGs by the novel laser-IGC technique,but also makes a great contribution to developing low-cost,high-efficient,and super-durable HER electrocat-alysts in acidic environment.

关键词： Nanoglass Inert gas condensations High energy states Hydrogen evolution reactions

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Multi-omics analyses provide insights into the evolutionary history and the synthesis of medicinal components of the Chinese wingnut

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Plant Diversity 2024年第3期46卷 309-320页

作者： Zi-yan Zhang He-Xiao Xia Meng-Jie Yuan feng Gao Wen-hua Bao Lan Jin Min Li Yong Li College of Life Science and Technology Inner Mongolia Normal UniversityHohhot 010020China Key Laboratory of Biodiversity Conservation and Sustainable Utilization in Mongolian Plateau for College and University of Inner Mongolia Autonomous Region Hohhot 010022China College of Landscape and Art Henan Agricultural UniversityZhengzhou 450002China State Key Laboratory of Tree Genetics and Breeding Chinese Academy of ForestryBeijing 100091China

Chinese wingnut(Pterocarya stenoptera)is a medicinally and economically important tree species within the family ***,the lack of high-quality reference genome has hindered its in-depth *** this study,we successfully assembled its chromosome-level genome and performed multiomics analyses to address its evolutionary history and synthesis of medicinal components.A thorough examination of genomes has uncovered a significant expansion in the Lateral Organ Boundaries Domain gene family among the winged group in *** notable increase may be attributed to their frequent exposure to flood-prone *** further differentiation between Chinese wingnut and Cyclocarya paliurus,significant positive selection occurred on the genes of NADH dehydrogenase related to mitochondrial aerobic respiration in Chinese wingnut,enhancing its ability to cope with waterlogging *** genomic analysis revealed Chinese wingnut evolved more unique genes related to arginine synthesis,potentially endowing it with a higher capacity to purify nutrient-rich water *** of terpene synthase families enables the production of increased quantities of terpenoid volatiles,potentially serving as an evolved defense mechanism against herbivorous *** combined transcriptomic and metabolomic analysis,we identified the candidate genes involved in the synthesis of terpenoid *** study offers essential genetic resources for Chinese wingnut,unveiling its evolutionary history and identifying key genes linked to the production of terpenoid volatiles.

关键词： Genome Medicinal components Metabolome Pterocarya stenoptera Transcriptome

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Dissolution kinetics and reaction mechanism of Al_(2)O_(3) in molten CaF_(2)-CaO-Al_(2)O_(3) slag

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Journal of Iron and Steel Research International 2024年第4期31卷 861-869页

作者： Yao-xin Du yan-wu Dong Zhou-hua Jiang Ganna Stovpchenko Yu-shuo Li Jun huang Xin-wei Wang Yu-xiao Liu School of Metallurgy Northeastern UniversityShenyang 110819LiaoningChina Key Laboratory of Ecological Metallurgy of Multimetallic Mineral Northeastern UniversityShenyang 110819LiaoningChina State Key Laboratory of Rolling and Automation Northeastern UniversityShenyang 110819LiaoningChina E.O.Paton Electric Welding Institute of the National Academy of Science of Ukraine Kiev 03115Ukraine

The dissolution behavior of Al_(2)O_(3) in molten CaF_(2)-CaO-Al_(2)O_(3) slag,a basic slag system of electroslag remelting process,was investigated by rotating cylinder method using corundum rods to simulate Al_(2)O_(3) inclusions in *** experimental results show that the dissolution rate of Al_(2)O_(3);rods in CaF_(2)-CaO-Al_(2)O_(3) slag increases with the increase in rotating speed and temperature,and the rate-controlling step is the mass transfer in the *** dissolution rate of Al_(2)O,in CaF_(2)-CaO-Al_(2)O_(3) slag increases with the increase in the ratio of CaO to Al_(2)O_(3),which is due to the increase in dissolution driving force and the decrease in slag *** apparent activation energy of the mass transfer of AlOs in slag C is calculated to be 222.86 kJ mol^(-1).During the dissolution of Al_(2)0,inclusions in the slag,it reacts with F in liquid slag at first,then reacts with CaO to form the intermediate compounds of xCaO-yAl_(2)O_(3) system,and finally dissolves in molten *** dissolution rate of Al_(2)O_(3) inclusions in CaF_(2)-CaO-Al_(2)O_(3) slag for electroslag remelting is positively correlated with the ratio of the dissolution driving force and slag viscosity,and the correlation coeficient is 2.487×10^(-11).

关键词： Inclusion Electroslag remelting Mass transfer:Dissolution rate-Dissolution driving force

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