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Relationship of familial cytochrome P450 4V2 gene mutation with liver cirrhosis:A case report and review of the literature

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World Journal of Clinical Cases 2022年第28期10卷 10346-10357页

作者： Jin-Lian Jiang Jiang-Fu Qian de-Hui Xiao Xia Liu Fang Zhu Jie Wang Zhou-Xiong Xing de-lin xu Yuan xue Yi-Huai He department of Infectious Diseases Affiliated Hospital of Zunyi Medical UniversityZunyi 563003Guizhou ProvinceChina department of Digestion Dafang County People’s HospitalBijie 551600Guizhou ProvinceChina department of Intensive Care Affiliated Hospital of Zunyi Medical UniversityZunyi 563000Guizhou ProvinceChina department of Cell Biology Zunyi Medical UniversityZunyi 563099Guizhou ProvinceChina department of Liver Diseases the Third People’s Hospital of ChangzhouChangzhou 213000Jiangsu ProvinceChina

BACKGROUND Many genetic and metabolic diseases affect the liver,but diagnosis can be difficult because these diseases may have complex clinical manifestations and diverse clinical *** is also incomplete clinical knowledge of these many different diseases and limitations of current testing *** SUMMARY We report a 53-year-old female from a rural area in China who was hospitalized for lower limb edema,abdominal distension,cirrhosis,and *** excluded the common causes of liver disease(drinking alcohol,using traditional Chinese medicines,hepatitis virus infection,autoimmunity,and hepatolenticular degeneration).When she was 23-years-old,she developed night-blindness that worsened to complete blindness,with no obvious *** parents were first cousins,and both were *** of the patient’s family history indicated that all 5 siblings had night blindness and impaired vision;one sister was completely blind;and another sister had night-blindness complicated with cirrhosis and subclinical *** exome sequencing showed that the patient,parents,and siblings all had mutations in the cytochrome P450 4V2gene(CYP4V2).The CYP4V2 mutations of the parents and two sisters were heterozygous,and the others were *** siblings also had heterozygous dual oxidase activator 2(DUOXA2) *** Mutations in the CYP4V2 gene may affect lipid metabolism and lead to chronic liver injury,fibrosis,and cirrhosis.

关键词： Cirrhosis Genetic metabolic liver disease Cytochrome P4504V2 Dual Oxidase activator 2 Bietti Crystalline corneoretinal dystrophy Case report

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Boundary Behaviors for a Continuous-state Nonlinear Neveu’s Branching Process

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Acta Mathematica Sinica,English Series 2024年第8期40卷 2005-2016页

作者： lin Yu BAI xu YANG School of Mathematics and Information Science North Minzu UniversityYinchuan 750021P.R.China

By generalizing a criterion of Mufa Chen for Markov jump processes,we establish the necessary and sufficient conditions for the extinction,explosion and coming down from infinity of a continuous-state nonlinear Neveu... 详细信息

关键词： Continuous-state branching process nonlinear branching Neveu’s branching extinction explosion coming down from infinity

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Effect of hot isostatic pressure on the microstructure and tensile properties of γ'-strengthened superalloy fabricated through induction-assisted directed energy deposition

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International Journal of Minerals,Metallurgy and Materials 2024年第5期31卷 1089-1097页

作者： Jianjun xu Hanlin Ding Xin lin Feng Liu Analytical&Testing Center Northwestern Polytechnical UniversityXi’an 710072China State Key Laboratory of Solidification Processing Northwestern Polytechnical UniversityXi’an 710072China

The microstructure characteristics and strengthening mechanism of Inconel738LC(IN-738LC) alloy prepared by using induction-assisted directed energy deposition(IdeD) were elucidated through the investigation of samples subjected to IdeD under 1050℃ preheating with and without hot isostatic pressing(HIP,1190℃,105 MPa,and 3 h).Results show that the as-deposited sample mainly consisted of epitaxial columnar crystals and inhomogeneously distributed γ’ phases in interdendritic and dendritic core *** HIP,grain morphology changed negligibly,whereas the size of the γ’ phase became increasingly *** further heat treatment(HT,1070℃,2 h + 845℃,24 h),the γ’ phase in the as-deposited and HIPed samples presented a bimodal size distribution,whereas that in the as-deposited sample showed a size that remained *** comparison of tensile properties revealed that the tensile strength and uniform elongation of the HIP + HTed sample increased by 5% and 46%,respectively,due to the synergistic deformation of bimodal γ’phases,especially large cubic γ’ ***,the relationship between phase transformations and plastic deformations in the Ideded sample was discussed on the basis of generalized stability theory in terms of the trade-off between thermodynamics and kinetics.

关键词： directed energy deposition Ni-based superalloys high-temperature preheating hot isostatic pressing microstructure tensile properties

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Thermal decomposition and Oxidative decomposition Mechanism of HFC-134a by Experimental and DFT Method

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Journal of Thermal Science 2024年第5期33卷 1990-2003页

作者： xu Yunting ZHANG Kai DAI Xiaoye SHI lin Key Laboratory for Thermal Science and Power Engineering of Ministry of Education Department of Energy and Power EngineeringTsinghua UniversityBeijing 100084China

In response to the Kigali Amendment to the Montreal Protocol and global low-carbon emission environmental requirements,the phase-out and decomposition of numerous HFC refrigerants have become urgent,necessitating efficient and mild decomposition *** study investigates the thermal decomposition and oxidative thermal decomposition pathways of the typical hydrofluorocarbon refrigerant HFC-134a,employing a combination of experimental and quantum chemical DFT simulation *** chemical simulations reveal that the initial reaction bond cleavage serves as the rate-determining step during the thermal decomposition process,with the most easily detectable closed-shell products including CF_(2)=CHF,HF,CH_(3)F,CHF_(3),CH_(2)F_(2),and CF_(4).Reactive oxygen species can significantly reduce the Gibbs free energy barrier for HFC-134a *** achieve efficient degradation of HFC-134a,appropriate catalysts should be developed and selected to increase the level of reactive oxygen species in the reaction *** studies further corroborate that HFC-134a may undergo degradation through distinct reaction pathways under varying temperature(240℃to360℃)and pressure(0.1 MPa to 4.5 MPa)conditions,in agreement with simulation predictions.

关键词： HFC-134a pyrolysis oxidative thermal decomposition DFT refrigerant

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Monitoring the energy variation of an electron linac using a Cerenkov detector

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Chinese Physics C 2010年第1期34卷 126-130页

作者： LI Shu-Wei WANG Yi LI Jin LI Yuan-Jing KANG Ke-Jun lin de-xu ZHANG Qing Jun department of Engineering Physics Tsinghua University Key Laboratory of Particle & Radiation Imaging (Tsinghua University) Ministry of Education Nuctech Company Limited

A new method to monitor the energy variation of a multi-energy electron linac by combining a Cerenkov detector and a CsI（Tl） detector is reported. The signals in the Cerenkov detector show an appreciable but different dependence on the energy of the electron linac from the traditional CsI（Tl） detector due to the particular response of the former to charged electrons with high velocity above threshold. The method is more convenient than the HVL （half-value layer） method which is commonly employed to calibrate the energy of an electron linac for real time monitoring. The preliminary validity of the method is verified in a dual-energy electron linac with 6 MeV and 3 MeV gears. Moreover, the method combining the Cerenkov detector and the CsI（Tl） detector is applicable to probe the X-ray spectrum hardened by the inspected material and may serve as a novel tool for material discrimination with effective atomic number in radiation imaging.

关键词： electron linac Cerenkov detector Geant4

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Transverse mode-coupling instability with longitudinal impedance

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Nuclear Science and Techniques 2024年第6期35卷 40-53页

作者： Hai-Sheng xu Chun-Tao lin Na Wang Jing-Ye xu Yuan Zhang Key Laboratory of Particle Acceleration Physics and Technology Institute of High Energy PhysicsChinese Academy of SciencesBeijing 100049China University of Chinese Academy of Sciences Beijing 100049China Institute of Advanced Science Facilities Shenzhen 518107GuangdongChina

Transverse mode-coupling instability(TMCI)is a dangerous transverse single-bunch instability that can lead to severe par-ticle *** mechanism of TMCI can be explained by the coupling of transverse coherent oscillation modes owing to the transverse short-range wakefield(i.e.,the transverse broadband impedance).Recent studies on future circular colliders,e.g.,FCC-ee,showed that the threshold of TMCI decreased significantly when both longitudinal and transverse impedances were *** performed computations for the circular electron-positron collider(CEPC)and observed a similar *** studies on the influence of longitudinal impedance on the TMCI threshold were *** concluded that the imaginary part of the longitudinal impedance,which caused a reduction in the incoherent synchrotron tune,was the primary reason for the reduction in the TMCI ***,the real part of the longitudinal impedance assists in increasing the TMCI threshold.

关键词： Transverse mode-coupling instability Longitudinal impedance

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Evaluation of suitability,adaptability,and reserve potential of construction land on the Qinghai-Tibet Plateau

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Journal of Geographical Sciences 2024年第1期34卷 41-61页

作者： YANG Hua xu Yong ZHOU Kan WANG Lijia xu lin Key Laboratory of Regional Sustainable development Modeling Institute of Geographic Sciences and Natural Resources ResearchCASBeijing 100101China University of Chinese Academy of Sciences Beijing 100049China

Construction land is the leading carrier of human activities such as production and *** the construction land suitability(CLS)on the Qinghai-Tibet Plateau(QTP)holds significant implications for harmonizing the relationship between ecological protection and human activity and promoting population and industry layout ***,no relevant studies provide a complete CLS assessment of the *** this study,we developed a model-based CLS evaluation framework coupling of pattern and process to calculate the global CLS on the QTP based on a previously developed CLS evaluation ***,using the land-use data of 1990,2000,2010,and 2020,we examined the adaptability of existing construction land(ECL)to the CLS assessment result through the adaptability index and vertical gradient index and further analyzed the limitations of maladaptive construction ***,we calculated the potential area of reserve suitable construction *** article includes four conclusions:(1)The highly suitable,suitable,moderately suitable,marginally suitable,and unsuitable CLS classes cover areas of 0.33×10^(4)km^(2),10.42×10^(4)km^(2),18.06×10^(4)km^(2),24.12×10^(4)km^(2),and 205.29×10^(4)km^(2),*** approximately 11%of the study area on the QTP is suitable for large-scale permanent construction land,and approximately 79.50%of the area is unsuitable under current economic and technological conditions.(2)The ECL adaptability index is 85.16%,85.93%,85.18%,and 78.01%during 1990–2020,respectively,with an average adaptability index exceeding 80%on the *** ECL distribution generally conforms to construction land suitable space characteristics but with a significant spatial difference.(3)From 1990 to 2020,the maladaptive ECL was dominated by rural settlement land,transport land,and special land,with a rapidly increasing proportion of urban and other construction *** maladaptive ECL is constrained by both elevation and slope in the southern Qinghai Pla

关键词： land resource construction land suitability existing construction land adaptability human activity Qinghai-Tibet Plateau

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Chinese Journal of Electronics 2024年第3期33卷 683-693页

作者： Jiamei LIU lin TAN Hong xu PLA Strategic Support Force Information Engineering University

deoxys-BC is the primitive tweakable block cipher of the deoxys family of authenticated encryption *** on existing related-tweakey boomerang distinguishers,this paper improves the boomerang attacks on 11-round deoxys-BC-256 and 13-round deoxys-BC-384 by the optimized key guessing and the precomputation *** transfers a part of subtweakey guess in the key-recovery phase to the precomputation resulting in a significant reduction of the overall time *** 11-round deoxys-BC-256,we give a related-tweakey boomerang attack with time/data/memory complexities of 2218.6/2125.7/2125.7,and give another attack with the less time complexity of2215.8and memory complexity of 2120 when the adversary has access to the full *** 13-round deoxys-BC-384,we give a related-tweakey boomerang attack with time/dat a/memory complexities of 2k-96+2157.5/2120.4/*** the key size k=256,it reduces the time complexity by a factor of 231compared with the previous 13-round boomerang *** addition,we present two new related-tweakey boomerang distinguishers on 11-round deoxys-BC-384 with the same probability as the best previous distinguisher.

关键词： Ciphers Memory management Complexity theory Encryption Time complexity

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Field Study of HPTRM Combined with Vegetation and Anchor to Protect Newly Excavated Expansive Soil Slope

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Journal of Earth Science 2024年第4期35卷 1277-1288页

作者： Yingzi xu xuhang Liao linqiang Tang lin Li College of Civil&Architectural Engineering Guangxi UniversityNanning530004China Guangxi Key Laboratory of Disaster Prevention and Engineering Safety Nanning530004China Guangxi Transport Vocational and Technical College Nanning530004China department of Civil&Architectural Engineering Tennessee State UniversityTennessee37209NashvilleUSA

Anchor reinforced vegetation system(ARVS)comprises high performance turf reinforcement mats(HPTRM),vegetation and *** is a new attempt to apply the system in expansive soil slope *** goal of this paper was to evaluate the effectiveness of ARVS in protecting newly excavated expansive soil *** field tests on the bare slope,grassed slope and ARVS protective slope were carried out,including natural and artificial *** the test,the soil water content,soil deformation,and anchor axial force were monitored,and then the slope protection mechanism of ARVS was *** was found that ARVS can effectively protect expansive soil slopes compared with bare slopes and grassed *** vegetation and HPTRM form a reinforced turf,and the anchors fix it to the slope surface,thus restraining the expansion *** axial force on the anchor of ARVS includes frictional resistance and tensile force transmitted by HPTRM,which is maximum at the early stage of *** neutral point of the anchor of ARVS moves deeper under atmospheric action,but the vegetation and HPTRM on the slope surface can limit this movement.

关键词： expansive soil slope anchor reinforced vegetation system high performance turf reinforcement mats field test slope protection

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Catalytically generated noncovalent ammonium dienolate:a versatile platform for the development of organocatalytic asymmetric cascade reactions

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Science China Chemistry 2024年第8期67卷 2524-2546页

作者： Jun-Bing lin Dong-Sheng Ji Peng-Fei xu State Key Laboratory of Applied Organic Chemistry College of Chemistry and Chemical EngineeringLanzhou UniversityLanzhou 730000China Shaanxi Key Laboratory of Chemical Reaction Engineering College of Chemistry and Chemical EngineeringYan’an UniversityYan’an 716000China

Organocatalytic cascade reactions represent a powerful strategy for the rapid construction of complex chiral molecules with multiple stereocenters from simple substrates under mild conditions. The intriguing structural feature and diverse reactivity of catalytically generated dienolate species render them competent and versatile intermediates for the development of practical and valuable cascade reactions. Over the past years, a plethora of innovative and pioneering noncovalent ammonium dienolatemediated cascade reactions have been designed and implemented under the catalysis of chiral organocatalysts, making dienolate activation a general, robust, and complementary method for the functionalization of unsaturated carbonyl compounds and related substances. This review illustrates the recent advances in organocatalytic noncovalent ammonium dienolate-mediated cascade reactions(mainly from 2010 to 2023), including the cascade transformations of ammonium dienolates directly generated from unsaturated ketone/aldehyde, ester/lactone/azlactone, amide/lactam/pyrazolone/oxindole, and alkylidene nitrile compounds. The contents are arranged based on the reaction types of the ammonium dienolates, with an emphasis on cascade 2,5-, 3,5-, and 4,5-difunctionalizations of these intermediates. Furthermore, other cascade reactions involving the 1,3-, 2,3-, and even more complex 3,4,5-reactivities of ammonium dienolates were also discussed. The reaction pathway, reaction stereoinduction, and synthetic applications of the ammonium dienolate-mediated cascade reactions were highlighted throughout the article. As a stimulating and ever-growing research area, the organocatalytic noncovalent ammonium dienolate-mediated cascade reactions are expected to continue demonstrating their magic power for constructing chiral targets in the future and further expanding the boundaries of asymmetric catalysis.

关键词： organocatalysis asymmetric catalysis ammonium dienolate cascade reaction difunctionalization

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