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Long-term clinical outcomes and health-related quality of life in patients with isolated methylmalonic acidemia after liver transplantation:experience from the largest cohort study in China

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World Journal of Pediatrics 2024年第8期20卷 809-821页

作者： Yi-Zhou Jiang Guang-peng Zhou Lin Wei Wei Qu zhi-Gui Zeng Ying Liu Yu-Le Tan Jun Wang zhi-Jun Zhu Li-Ying Sun Department of Critical Liver Diseases Liver Research CenterBeijing Friendship HospitalCapital Medical UniversityNo.101 Lu Yuan Dong RoadTong-Zhou DistrictBeijing110112China Liver Transplantation Center National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijingChina Clinical Center for Pediatric Liver Transplantation No.101 Lu Yuan Dong RoadTong-Zhou DistrictCapital Medical UniversityBeijing110112China

Background Liver transplantation(LT)has been proposed as a viable treatment option for selected methylmalonic acidemia(MMA)***,there are still controversies regarding the therapeutic value of LT for *** systematic assessment of health-related quality of life(HRQoL)-targeted MMA children before and after LT is also *** study aimed to comprehensively assess the long-term impact of LT on MMA,including multiorgan sequelae and HRQoL in children and *** We retrospectively evaluated 15 isolated MMA patients undergoing LT at our institution between June 2.13 and March ***-and post-transplant data were compared,including metabolic profiles,neurologic consequences,growth parameters,and *** further assess the characteristics of the HRQoL outcomes in MMA,we compared the results with those of children with biliary atresia(BA).Results All patients had early onset MMA,and underwent LT at a mean age of 4.3 *** 1.3–8.2.years of follow-up,the patient and graft survival rates were 100%.Metabolic stability was achieved in all patients with liberalized dietary protein *** was a significant overall improvement in height Z scores(P=0.0047),and some preexisting neurological complications remained stable or even improved after *** the Pediatric Quality of Life Inventory(PedsQL™)generic core scales,the mean total,physical health,and psychosocial health scores improved significantly posttransplant(P<0.05).In the family impact module,higher mean scores were noted for all subscales post-LT,especially family function and daily activities(P<0.01).However,the total scores on the generic core scales and transplant module were significantly lower(Cohen’s d=0.57–1.17)when compared with BA *** particular,social and school functioning(Cohen’s d=0.86–1.76),treatment anxiety,and communication(Cohen’s d=0.99–1.81)were far behind,with a large effect *** This large single-center study of the mainland of China sh

关键词： Health-related quality of life.Inborn error of metabolism Isolated methylmalonic acidemia Liver transplantation:Outcomes

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Advancements in machine learning for material design and process optimization in the field of additive manufacturing

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China Foundry 2024年第2期21卷 101-115页

作者： Hao-ran Zhou Hao Yang Huai-qian Li Ying-chun Ma Sen Yu Jian shi Jing-chang Cheng peng Gao Bo Yu zhi-quan Miao Yan-peng Wei National Key Laboratory of Advanced Casting Technologies Shenyang Research Institute of Foundry Co.Ltd.CAMShenyang 110022China Department of Mechanical Engineering Tsinghua UniversityBeijing 100084China Key Laboratory of Space Physics Beijing 100076China School of Materials Science and Engineering Nanyang Technological University639798Singapore

Additive manufacturing technology is highly regarded due to its advantages,such as high precision and the ability to address complex geometric ***,the development of additive manufacturing process is constrained by issues like unclear fundamental principles,complex experimental cycles,and high *** learning,as a novel artificial intelligence technology,has the potential to deeply engage in the development of additive manufacturing process,assisting engineers in learning and developing new *** paper provides a comprehensive overview of the research and applications of machine learning in the field of additive manufacturing,particularly in model design and process ***,it introduces the background and significance of machine learning-assisted design in additive manufacturing *** then further delves into the application of machine learning in additive manufacturing,focusing on model design and process ***,it concludes by summarizing and forecasting the development trends of machine learning technology in the field of additive manufacturing.

关键词： additive manufacturing machine learning material design process optimization intersection of disciplines embedded machine learning

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Clinical features and possible pathogenesis of multiple evanescent white dot syndrome with different retinal diseases and events:a narrative review

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International Journal of Ophthalmology(English edition) 2024年第3期17卷 583-595页

作者： Chun-Li Chen Yi-Zhe Cheng zhi-Han Zhang Ge Wang Xiao-Yan peng Department of Ophthalmology Beijing Tongren HospitalCapital Medical UniversityBeijing 100730China Beijing Ophthalmology and Visual Science Key Laboratory Beijing 100730China State Key Laboratory of Ophthalmology Zhongshan Ophthalmic CenterSun Yat-sen UniversityGuangzhou 510600Guangdong ProvinceChina

●Multiple evanescent white dot syndrome(MEWDS)is a rare fundus disease,characterized by acute vision loss and visual field *** previous studies have explained the possible pathogenesis and clinical features of primary ***,as the number of reported cases increases,secondary MEWDS occurs in other related retinal diseases and injuries,exhibiting some special *** associated retinal diseases include multifocal choroiditis/punctate inner choroidopathy(MFC/PIC),acute zonal occult outer retinopathy,best vitelliform macular dystrophy,pseudoxanthoma elasticum,and ocular *** related retinal injury is laser photocoagulation,surgery,and *** primary MEWDS often have a self-limiting course,secondary MEWDS may require treatment in some cases,according to the severity of concomitant diseases and ***,MEWDS secondary to MFC/PIC that is prone to forming choroidal neovascularization and focal choroidal excavation,needs positive treatment with *** possible underlying pathogenesis of secondary MEWDS is the exposure of choroidal antigen after the disruption of Bruch’s *** MEWDS-related features in secondary MEWDS are still evanescent under most *** prognosis and treatment depend on the severity of *** studies propose that the etiology is associated with immune factors,including viral infection,inflammation in choroid and Bruch’s membrane,and antigen exposure caused by retinal and/or choroidal *** pathogenic studies should be conducted in the *** diagnosis for secondary MEWDS could benefit patients in aspects of management and prognosis.

关键词： multiple evanescent white dot syndrome punctate inner choroidopathy multifocal choroiditis secondary multiple evanescent white dot syndrome

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In vivo imaging reveals a synchronized correlation among neurotransmitter dynamics during propofol and sevoflurane anesthesia

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Zoological Research 2024年第3期45卷 679-690页

作者： Gao-Lin Qiu Li-Jun peng peng Wang {2.-Lai Yang Ji-Qian Zhang Hu Liu Xiao-Na Zhu Jin Rao Xue-Sheng Liu Department of Anesthesiology First Affiliated Hospital of Anhui Medical UniversityHefeiAnhui 230022China School of Life Science and Technology ShanghaiTech UniversityShanghai 201210China

General anesthesia is widely applied in clinical ***,the precise mechanism of loss of consciousness induced by general anesthetics remains ***,we measured the dynamics of five neurotransmitters,includingγ-aminobutyric acid,glutamate,norepinephrine,acetylcholine,and dopamine,in the medial prefrontal cortex and primary visual cortex of C57BL/6 mice through in vivo fiber photometry and genetically encoded neurotransmitter sensors under anesthesia to reveal the mechanism of general anesthesia from a neurotransmitter *** revealed that the concentrations of γ-aminobutyric acid,glutamate,norepinephrine,and acetylcholine increased in the cortex during propofol-induced loss of *** levels did not change following the hypnotic dose of propofol but increased significantly following surgical doses of propofol ***,the concentrations of the five neurotransmitters generally decreased during sevoflurane-induced loss of ***,the neurotransmitter dynamic networks were not synchronized in the non-anesthesia groups but were highly synchronized in the anesthetic *** findings suggest that neurotransmitter dynamic network synchronization may cause anesthetic-induced loss of consciousness.

关键词： General anesthesia Loss of consciousness In vivo neurotransmitter imaging Medial prefrontal cortex Primary visual cortex

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Graphene-anchored sodium single atoms:A highly active and stable catalyst for transesterification reaction

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Nano Research 2024年第6期17卷 4979-4985页

作者： Song-Song peng Yao Nian Xing-Ru Song Xiang-Bin Shao Chen Gu zhi-Wei Xing Shi-Chao Qi peng Tan You Han Xiao-Qin Liu Lin-Bing Sun State Key Laboratory of Materials-Oriented Chemical Engineering Jiangsu National Synergetic Innovation Center for Advanced Materials(SICAM)College of Chemical EngineeringNanjing Tech University30 South Puzhu RoadNanjing 211816China School of Chemical Engineering and Technology Tianjin UniversityTianjin 300072China Haihe Laboratory of Sustainable Chemical Transformations Tianjin 300192China

Solid strong base catalysts have received considerable attention in various organic reactions due to their facile separation,neglectable corrosion,and environmental *** great progress has been made in the preparation of solid strong base catalysts,it is still challenging to avoid basic sites aggregation on support and active sites loss in reaction ***,we report a tandem redox strategy to prepare Na single atoms on graphene,producing a new kind of solid strong base catalyst(Na1/G).The base precursor NaNO_(3)was first reduced to Na2. by graphene(400℃)and successively to single atoms Na anchored on the graphene vacancies(800℃).Owing to the atomically dispersed of basicity,the resultant catalyst presents high activity toward the transesterification of methanol and ethylene carbonate to synthesize dimethyl carbonate(turnover frequency(TOF)value:12..7 h^(−1)),which is much better than the conventional counterpart Na2./G and various reported solid strong bases(TOF:1.0-90.1 h^(−1)).Furthermore,thanks to the basicity anchored on graphene,the Na1/G catalyst shows excellent durability during *** work may provide a new direction for the development of solid strong base catalysts.

关键词： solid strong base catalyst basic sites redox graphene single Na atoms

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Numerical evaluations on the fluid production in the in-situ conversion of continental shale oil reservoirs

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Petroleum Science 2024年第4期21卷 2485-2501页

作者： Zhao-Bin Zhang Maryelin Josefina Brice?o Montilla Shou-Ding Li Xiao Li Jian-peng Xing Yan-zhi Hu Key Laboratory of Shale Gas and Geoengineering Institute of Geology and Geophysics Chinese Academy of Sciences Institute of Earth Sciences Chinese Academy of Sciences College of Earth and Planetary Sciences University of Chinese Academy of Sciences Institute of Energy Peking University

In-situ conversion presents a promising technique for exploiting continental oil shale formations,characterized by highly fractured organic-rich rock. A 3D in-situ conversion model, which incorporates a discrete fracture network, is developed using a self-developed thermal-flow-chemical(TFC) *** of the model elucidates the in-situ conversion process in three stages and defines the transformation of fluids into three distinct outcomes according to their end stages. The findings indicate that kerogen decomposition increases fluid pressure, activating fractures and subsequently enhancing permeability. A comprehensive analysis of activated fracture permeability and heating power reveals four distinct production modes, highlighting that increasing heating power correlates with higher cumulative fluid production. Activated fractures, with heightened permeability, facilitate the mobility of heavy oil toward production wells but hinder its cracking, thereby limiting light hydrocarbon ***, energy efficiency research demonstrates the feasibility of the in-situ conversion in terms of energy utilization, especially when considering the surplus energy from high-fluctuation energy sources such as wind and solar power to provide heating.

关键词： In-situ conversion Continental shale oil Natural fracture network TFC model

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The occurrence characteristics of oil in shales matrix from organic geochemical screening data and pore structure properties:An experimental study Author links open overlay panel

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Petroleum Science 2024年第1期21卷 1-13页

作者： Zi-zhi Lin Jun-Qian Li Shuang-Fang Lu Qin-Hong Hu peng-Fei Zhang Jun-Jie Wang Qi zhi Hong-Sheng Huang Na Yin Yue Wang Tian-Chen Ge National Key Laboratory of Deep Oil and Gas China University of Petroleum(East China)Qingdao 266580ShandongChina School of Geosciences China University of Petroleum(East China)Qingdao 266580ShandongChina Sanya Offshore Oil&Gas Research Institute Northeast Petroleum UniversitySanya 572025HainanChina College of Earth Science and Engineering Shandong University of Science and TechnologyQingdao 266590ShandongChina

The occurrence characteristics of shale oil are of great significance to the movability of shale *** this study,the occurrence characteristics of oil in the shale matrix at Funing Formation shale in Subei Basin were quantitatively evaluated by organic geochemistry and microscopic pore structure characterization *** Multiple Isothermal Stages Pyrolysis(MIS)experiment results show that the content of total oil,adsorbed oil,and free oil in the shales are 3.15-11.2. mg/g,1.41-4.95 mg/g,and 1.74-6.51 mg/g,*** which the silicon-rich shale has the best *** relative content of free oil shows an increasing trend in pores with pore diameters greater than 3 *** the relative content of free oil reaches 100%,the pore size of silicon-rich shale is about 2.0 nm,while that of calcium-rich shale,clay-rich shale,and siliceous mixed shale is about 10 *** occurrence law of adsorbed oil is opposite to that of free oil,which indicates that shale oil will occur in the pores and fractures in a free state in a more extensive pore size range(>2.0 nm).This study also enables us to further understand the occurrence characteristics of shale oil under the interaction of occurrence state and occurrence space.

关键词： Shale oil Occurrence characteristics Sequential solvent extraction Low-temperature nitrogen adsorption Funing Formation Dongtai Depression

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Flotation separation of feldspar from quartz using sodium fluosilicate as a selective depressant

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Rare Metals 2024年第3期43卷 1288-1300页

作者： Xin Hu Xian-Ping Luo Zi-Shuai Liu Yong-Bing Zhang He-peng Zhou zhi-Zhao Yang Xue-Kun Tang Faculty of Resource and Environmental Engineering Jiangxi University of Science and TechnologyGanzhou341000China Yichun Lithium New Energy Industry Research Institute Jiangxi University of Science and TechnologyYichun336000China

The physical and chemical properties of feldspar and quartz are highly similar,thus they cannot be easily separated *** this work,the flotation separation of feldspar from quartz using sodium fluosilicate(Na_(2.SiF_(6);SF)as a selective depressant was ***,the influence mechanism of SF on the selective flotation separation of feldspar and quartz was investigated via flotation tests,chemical analysis of flotation solution,Fourier transform infrared spectroscopy(FTIR),X-ray photoelectron spectroscopy(XPS)and contact angle *** results of the flotation tests show that feld spar and quartz without SF treatment have good *** SF treatment,feldspar still has good floatability,whereas quartz has significantly reduced *** solution chemical analysis shows that the functional group plays an important role in depressing the quartz is[SiF_(6)]^(2.).FTIR,XPS and contact angle analysis results show that[SiF_(6)]^(2.)is adsorbed only on the surface of ***,a thin hydrophilic SiOF layer is generated on the surface,which interferes with the adsorption of the collector on the surface of the *** phenomenon leads to a significant reduction in quartz's ***,SF has a good ability to separate feldspar from quartz by flotation.

关键词： Flotation separation Depressants Sodium fluorosilicate Quartz Feldspar

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A Remark on the Affine Coordinates for KdV Tau-Functions

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Chinese Quarterly Journal of Mathematics 2024年第3期39卷 324-330页

作者： FU zhi-peng University of Science and Technology of China Hefei 230026China

We give a proof of an explicit formula for affine coodinates of points in the Sato’s infinite Grassmannian corresponding to tau-functions for the KdV hierarchy.

关键词： Sato’s infinite Grassmannian KdV hierarchy Affine coordinates

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Alarm Log Data Augmentation Algorithm Based on a GAN Model and Apriori

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Journal of Computer Science & Technology 2024年第4期39卷 951-966页

作者： Yang Yang Yu-Ting Li Yong-Hua Huo zhi-peng Gao Lan-Lan Rui State Key Laboratory of Network and Switching Technology Beijing University of Posts and TelecommunicationsBeijing 100876China The 54th Research Institute of China Electronics Technology Group Corporation Shijiazhuang 050081China

The complexity of alarm detection and diagnosis tasks often results in a lack of alarm log *** to the strong rule associations inherent in alarm log data,existing data augmentation algorithms cannot obtain good results for alarm log *** address this problem,this paper introduces a new algorithm for augmenting alarm log data,termed APRGAN,which combines a generative adversarial network(GAN)with the Apriori *** generates alarm log data under the guidance of rules mined by the rule ***,we propose a new dynamic updating mechanism to alleviate the mode collapse problem of the *** addition to updating the real reference dataset used to train the discriminator in the GAN,we dynamically update the parameters and the rule set of the Apriori algorithm according to the data generated in each *** extensive experimentation on two public datasets,it is demonstrated that APRGAN surpasses other data augmentation algorithms in the domain with respect to alarm log data augmentation,as evidenced by its superior performance on metrics such as BLEU,ROUGE,and METEOR.

关键词： data augmentation alarm log data Apriori generative adversarial network(GAN)

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