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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 2013 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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Self-assembled high-entropy nitride multilayer coating

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Rare Metals 2024年第6期43卷 2876-2883页

作者： Xiang-Yu Zhang Yan liu Vasiliy Pelenovich Xiao-Mei zeng Jie liu Qiang Wan Alexander Pogrebnjak Long-Jian Xue zhi-Wen Chen Yan Lei Bing Yang School of Power and Mechanical Engineering Wuhan UniversityWuhan 430072China Hubei JiuFengShan Future Science and Technology Laboratory Wuhan 420000China Institute of Technological Sciences of Wuhan University Wuhan 430072China Hubei Key Laboratory of Electronic Manufacturing and Packaging Integration Wuhan UniversityWuhan 430072China College of Engineering Huazhong Agricultural UniversityWuhan 430070China Sumy State University Sumy 40007Ukraine Institute of Materials Science Slovak University of Technology in BratislavaJána Bottu2591724 TrnavaSlovak Republic Shenzhen Research Institute of Wuhan University Shenzhen 518057China

The development of advanced materials and construction of multilayer structure contribute to enhancing the performance of protective coatings,further improving the industrial productivity and conserving *** this paper,the self-assembled high-entropy nitride(HEN)AlCrNbSiTiN multilayer coating was designed by harnessing the plasma distribution characteristics and depos-ited by arc ion plating using single target.

关键词： coating energy multilayer

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Achieving strength-ductility synergy in novel paramagnetic Fe-based medium-entropy alloys through deep cryogenic deformation

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Rare Metals 2024年第9期43卷 4493-4507页

作者： Hu-Wen Ma Yan-Chun Zhao Li Feng Tian-zeng liu zhi-Qi Yu Bo Jin Wang-Chun Duan Peter K.Liaw Dong Ma State Key Laboratory of Advanced Processing and Recycling of Non-Ferrous Metals Lanzhou University of TechnologyLanzhou 730050China Wenzhou Pump and Valve Engineering Research Institute Lanzhou University of TechnologyWenzhou 325105China Iron and Steel Research Institute Jiuquan Iron and Steel CorporationJiayuguan 735100China Lanzhou Resources and Environment Voc-Tech University Lanzhou 730123China Department of Materials Science and Engineering The University of TennesseeKnoxvilleTN 37996-2200USA Neutron Science Center Songshan Lake Materials LaboratoryDongguan 523808China

Cryogenic pre-deformation treatment has been widely used to effectively improve the comprehensive mechanical properties of steels and novel ***,the dislocation evolution and phase transformation induced by different degrees of deep cryogenic deformation are not yet fully *** this study,the effects of multiple cryogenic pre-treatments on the mechanical properties and deformation mechanisms of a paramagnetic Fe_(63.3)Mn_(14-)Si_(9.1)Cr_(9.8)C_(3.8)medium-entropy alloy(MEA)were investigated,leading to the discovery of a pretreated MEA that exhibits exceptional mechanical properties,including a fracture strength of 3.0 GPa,plastic strain of 26.1%and work-hardening index of *** addition,X-ray diffraction(XRD)and transmission electron microscopy(TEM)analyses revealed that multiple cryogenic pre-deformation treatments significantly increased the dislocation density of the MEA(from 9×10^(15)to 4×10^(16)m^(-2)after three pretreatments),along with a transition in the dislocation type from predominantly edge dislocations to mixed dislocations(including screw-and edge-type dislocations).Notably,this pretreated MEA retained its paramagnetic properties(μ_(r)<1.0200)even after *** calculations showed that cryogenic pretreatment can significantly reduce the stacking fault energy of the MEA by a factor of approximately four(i.e.,from 9.7 to2.6 m J·m^(-2)),thereby activating the synergistic effects of transformation-induced plasticity,twinning-induced plasticity and dislocation strengthening *** synergistic effects lead to simultaneous strength and ductility enhancement of the MEA.

关键词： Deep cryogenic transformation Iron-based medium-entropy alloys Dislocation evolution Phase transformation Stacking fault energy

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Bioinspired design of integral molded Janus silk fibroin-MXene evaporator for efficient solar vapor generation

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Nano Research 2024年第4期17卷 2824-2835页

作者： Ling-Qing Kong zhi-Cheng zeng Guang-Zong Min Zhao-Hui Meng Guo-Qing Meng Xiao-Xiao Guo Nai-Bo Lin Xiang-Yang liu The Higher Educational Key Laboratory for Biomedical Engineering of Fujian Province Research Center of Biomedical Engineering of XiamenDepartment of BiomaterialsCollege of MaterialsXiamen UniversityXiamen 361005China The State Key Laboratory of Marine Environmental Science(MEL) College of Ocean and Earth SciencesXiamen UniversityXiamen 361005China

Solar vapor generation is a promising sustainable technology that uses solar distillation to produce fresh water from seawater and wastewater,helping relieve global water resource ***,inspired by naturally grown integrally molded mulberry leaves with a Janus hydrophilic and hydrophobic structure,a novel,simple,and efficient integrated molding method is proposed to break through the limitations of the traditional split manufacturing strategy and realizes the integrated formation of Janus *** on the spontaneous sedimentation characteristics of MXene in silk fibroin solution and its regulation of mesoscopic structure and hydrophilicity of silk fibroin,layered structures with different compositions and hydrophilicities were obtained in one ***,ethanol and glutaraldehyde were added to construct a physical crystallization-chemical crosslinking dual stabilization structure in silk *** evaporator has the evaporation rate of 3.07 kg·m^(-2)·h^(-1) and the efficiency of 86.8%under 1 sun and maintains high evaporation performance under various extreme test conditions including vigorous washing,repeated compression,and high-intensity ultraviolet(UV)***,the evaporator performs well in practical application scenarios,its evaporation rate in the simulated Dead Sea seawater exceeds 2.13 kg·m^(-2)·h^(-1),and more than 99.9%of the salt,heavy metal ions,oil pollution,and dyes are purified.

关键词： silk fibroin MXene bioinspired integrated molding Janus structure solar vapor generation

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A Low Cost, Green Sustainable and Biodegradable Biomass-based Fluorine-free Water and Oil Repellent

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Chinese Journal of Polymer Science 2024年第4期42卷 480-491页

作者： zhi-Wei Shen Shu-Feng Hu Chen zeng Wei-Qing Kong Mei-Fang Zhu State Key Laboratory for Modification of Chemical Fibers&Polymer Materials College of Materials Science and EngineeringDonghua UniversityShanghai201620China State Key Laboratory of Pulp and Paper Engineering School of Light Industry and EngineeringSouth China University of TechnologyGuangzhou510630China

With the global ban on plastics intensifying, the substitution of plastic with paper has garnered increasing attention. However, the inadequate water and oil repellency of pulp molding hinders its practical applications. Currently, the common method to enhance the oil and water repellency of pulp molding is by adding fluorinated water and oil repellents. Nevertheless, fluorinated compounds are environmentally and physiologically harmful. Therefore, the development of fluorine-free, water and oil repellent alternatives is crucial. In this study, chitosan and stearic acid were utilized as the first and second layers of the oil and water repellent coatings, respectively. The coated samples exhibited favorable water repellency, with a water contact angle of 116.4°, and excellent oil repellency, achieving a 12/12 rating on the kit scale. Importantly, the samples did not exhibit any leakage after being soaked in hot water and hot oil at 95±5℃ for 30 min, demonstrating remarkable performance as a barrier against hot water and oil. Moreover, the coated samples displayed outstanding mechanical properties, thermal stability, biodegradability, and recyclability. The approach presented in this study is simple, cost-effective, environmentally friendly, and represents a promising technique for producing fluoride-free, oil-and water-resistant pulp molding products.

关键词： Biomass Fluoride-free Oil-andwater-resistant Plasticfree

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社会可持续发展与人的全面发展

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河北学刊 2008年第4期28卷 173-175页

作者：刘增芝保定学院马列部河北保定071000

可持续发展观是一种崭新的社会发展观,目的是寻求一条使人与自然得以和谐发展的道路,其最终内核和终极目的就是人的全面发展。社会的可持续发展与人的全面发展是相辅相成的。一方面,社会的可持续发展是人的全面发展的前提条件;另一方面... 详细信息

可持续发展观是一种崭新的社会发展观,目的是寻求一条使人与自然得以和谐发展的道路,其最终内核和终极目的就是人的全面发展。社会的可持续发展与人的全面发展是相辅相成的。一方面,社会的可持续发展是人的全面发展的前提条件;另一方面,人的全面发展为社会的可持续发展提供动力保障。

关键词：社会可持续发展科学发展观人的全面发展人的素质

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Highly Sensitive Ammonia Gas Sensors at Room Temperature Based on the Catalytic Mechanism of N,C Coordinated Ni Single-Atom Active Center

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Nano-Micro Letters 2024年第12期16卷 515-531页

作者： Wenjing Quan Jia Shi Min zeng Wen Lv Xiyu Chen Chao Fan Yongwei Zhang Zhou liu Xiaolu Huang Jianhua Yang Nantao Hu Tao Wang zhi Yang National Key Laboratory of Advanced Micro and Nano Manufacture Technology Shanghai Jiao Tong UniversityShanghai 200240People’s Republic of China Department of Micro/Nano Electronics School of Electronic Information and Electrical EngineeringShanghai Jiao Tong UniversityShanghai 200240People’s Republic of China Shanghai Key Laboratory of Intelligent Sensing and Detection Technology School of Mechanical and Power EngineeringEast China University of Science and TechnologyShanghai 200237People’s Republic of China

Significant challenges are posed by the limitations of gas sensing mechanisms for trace-level detection of ammonia(NH3).In this study,we propose to exploit single-atom catalytic activation and targeted adsorption properties to achieve highly sensitive and selective NH3 gas ***,Ni singleatom active sites based on N,C coordination(Ni-N-C)were interfacially confined on the surface of two-dimensional(2D)MXene nanosheets(Ni-N-C/Ti_(3)C_(2)Tx),and a fully flexible gas sensor(MNPE-Ni-N-C/Ti_(3)C_(2)Tx)was *** sensor demonstrates a remarkable response value to 5 ppm NH3(27.3%),excellent selectivity for NH3,and a low theoretical detection limit of 12.1 *** analysis by density functional calculation reveals that the Ni single-atom center with N,C coordination exhibits specific targeted adsorption properties for ***,its catalytic activation effect effectively reduces the Gibbs free energy of the sensing elemental reaction,while its electronic structure promotes the spill-over effect of reactive oxygen species at the gas-solid *** sensor has a dual-channel sensing mechanism of both chemical and electronic sensitization,which facilitates efficient electron transfer to the 2D MXene conductive network,resulting in the formation of the NH3 gas molecule sensing ***,the passivation of MXene edge defects by a conjugated hydrogen bond network enhances the long-term stability of MXene-based electrodes under high humidity *** work achieves highly sensitive room-temperature NH3 gas detection based on the catalytic mechanism of Ni single-atom active center with N,C coordination,which provides a novel gas sensing mechanism for room-temperature trace gas detection research.

关键词： Gas sensor Single atom Catalytic activation Targeted adsorption End-sealing passivation

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Enhanced activation of peroxymonosulfate on layered molybdenum disulfide loaded with Co-La bimetallic oxide for efficient degradation of chloramphenicol

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Rare Metals 2024年第9期43卷 4628-4635页

作者： Yu-Ning Fan Yun-Qiu Zhang San-Shuang Gao zeng-Hui Bi Yin Wang Shu-Xing Zhou Qian liu Tian-Ping Lv Guang-zhi Hu Institute for Ecological Research and Pollution Control of Plateau Lakes School of Ecology and Environmental ScienceYunnan UniversityKunming 650504China Hubei Key Laboratory of Low Dimensional Optoelectronic Materials and Devices Hubei University of Arts and ScienceXiangyang 441053China Institute for Advanced Study Chengdu UniversityChengdu 610106China

The use of transition metal-activated peroxymonosulfate(PMS)as an advanced oxidation technology has gained *** study developed a catalyst using cobaltDlanthanum bimetallic oxide supported on layered molybdenum disulfide(Mo S_(2))as a *** CoDLa/Mo S_(2)catalyst was synthesised through coprecipitation,followed by calcination with an optimised metal ratio of Co:La=2:1 to activate PMS and degrade trace chloramphenicol(CAP)in *** chemical composition of the catalyst was confirmed using X-ray diffraction(XRD)and X-ray photoelectron spectroscopy(XPS).At catalyst and PMS dosages of 0.1 and 0.5 g·L^(-1),respectively,the degradation rate of CAP was 95%with in 30 *** catalyst exhibitedstrong resistance to most interfering anions and maintained a high degradation rate at p H *** chromatographyDmass spectrometry analysis revealed the potential degradation pathways of CAP in the CoDLa/Mo S_(2)(2:1)/PMS *** other pollutants,such as oxytetracycline,complete degradation was achieved within 20 min,demonstrating the broad applicability of the CoDLa/Mo S_(2)(2:1)/PMS system for the degradation and removal of antibiotic organic contaminants.

关键词： catalyst molybdenum disulfide

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Learning Sequential and Structural Dependencies Between Nucleotides for RNA N6-Methyladenosine Site Identification

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IEEE/CAA Journal of Automatica Sinica 2024年第10期11卷 2123-2134页

作者： Guodong Li Bowei Zhao Xiaorui Su Dongxu Li Yue Yang zhi zeng Lun Hu Xinjiang Technical Institute of Physics and Chemistry Chinese Academy of ScienceUrumqi 830011China College of Computer Xi’an Jiaotong UniversityXi’an 710049China IEEE

N6-methyladenosine(m6A)is an important RNA methylation modification involved in regulating diverse biological processes across multiple ***,the identification of m6A modification sites provides valuable insight into the biological mechanisms of complex diseases at the post-transcriptional *** a variety of identification algorithms have been proposed recently,most of them capture the features of m6A modification sites by focusing on the sequential dependencies of nucleotides at different positions in RNA sequences,while ignoring the structural dependencies of nucleotides in their threedimensional *** overcome this issue,we propose a cross-species end-to-end deep learning model,namely CR-NSSD,which conduct a cross-domain representation learning process integrating nucleotide structural and sequential dependencies for RNA m6A site ***,CR-NSSD first obtains the pre-coded representations of RNA sequences by incorporating the position information into single-nucleotide states with chaos game representation *** then constructs a crossdomain reconstruction encoder to learn the sequential and structural dependencies between *** minimizing the reconstruction and binary cross-entropy losses,CR-NSSD is trained to complete the task of m6A site *** experiments have demonstrated the promising performance of CR-NSSD by comparing it with several state-of-the-art m6A identification ***,the results of cross-species prediction indicate that the integration of sequential and structural dependencies allows CR-NSSD to capture general features of m6A modification sites among different species,thus improving the accuracy of cross-species identification.

关键词： Cross-domain reconstruction cross-species prediction N6-methyladenosine(m6A)modification site RNA sequence sequential and structural dependencies

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Fu-Rec:Multi-Task Learning Recommendation Model Fusing Neighbor-Discrimination and Self-Discrimination

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Wuhan University Journal of Natural Sciences 2024年第2期29卷 134-144页

作者： ZHENG Sirui HUANG Bo liu Jin zeng Guohui YIN Ling LI zhi SUN Tie School of Electronic and Electrical Engineering Shanghai University of Engineering ScienceShanghai 201600China School of Computer Wuhan UniversityWuhan 430072HubeiChina School of Computer Science and Engineering Guangxi Normal UniversityGuilin 541004GuangxiChina AIoT Manufacturing Solutions Technology Co. Ltd.Hefei 230000AnhuiChina

In recent years,self-supervised learning has achieved great success in areas such as computer vision and natural language processing because it can mine supervised signals from unlabeled data and reduce the reliance on manual ***,the currently generated self-supervised signals are either neighbor discrimination or self-discrimination,and there is no model to integrate neighbor discrimination and *** on this,this paper proposes Fu-Rec that integrates neighbor-discrimination contrastive learning and self-discrimination contrastive learning,which consists of three modules:(1)neighbor-discrimination contrastive learning,(2)selfdiscrimination contrastive learning,and(3)recommendation *** neighbor-discrimination contrastive learning and selfdiscrimination contrastive learning tasks are used as auxiliary tasks to assist the recommendation *** Fu-Rec model effectively utilizes the respective advantages of neighbor-discrimination and self-discrimination to consider the information of the user’s neighbors as well as the user and the item itself for the recommendation,which results in better performance of the recommendation *** results on several public datasets demonstrate the effectiveness of the Fu-Rec proposed in this paper.

关键词： self-supervised learning recommendation system contrastive learning multi-task learning

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