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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

ZnO/ZnS heterostructure in N-doped porous carbon for anchoring-diffusion-conversion of polysulfides for high-performance lithium-sulfur batteries

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Rare Metals 2024年第8期43卷 3661-3676页

作者： Fei Wang Rong-Wei Huang Wen-Chang Han chun-Man Yang Wen-Hao Yang Peng Dong Chao-ling Wu Yi-Yong Zhang xue li ying-Jie Zhang National Local Joint Engineering Research Center for lithiumIon Batteries and Materials Preparation Technology Key Laboratory of Advanced Batteries Materials of Yunnan ProvinceFaculty of Metallurgical and Energy EngineeringKunming University of Science and TechnologyKunming 650093China Engineering Research Center of Alternative Energy Materials and devices Sichuan UniversityMinistry of EducationChengdu 610065China

The shuttle effect of polysulfides is one of the key factors hindering the commercialization of lithiumsulfur batteries(LSBs).Owing to their high conductivity and advantageous structure,heterostructures can be used in sulfur fixation and catalysis of *** this study,a flower-shaped ZnO/ZnS heterostructure on a nitrogendoped porous carbon(NPC) sulfur host was *** ZnO/ZnS heterostructure regulates the electronic structure of the material and exhibits higher metal-like ***,the ZnO/ZnS heterostructure combines the strong adsorption property of ZnO and the high catalytic ability of ZnS to realize the anchoring-diffusionconversion of lithium poly sulfides(liPSs).Results reveal that the developed ZnO/ZnS@NPC/S cathode has excellent electrochemical performance in LSBs,achieving a high discharge specific capacity of 1365.3 mAh·g^(-1) at 0.1C and excellent rate capability(719 mAh·g^(-1) at 2C;the capacity decay rate is only 0.042% per cycle after 1000 cycles).Even under a high sulfur loading-E/S(electrolyte/sulfur)ratio of 5.1 mg·cm^(-2)-6 μl·mg^(-1),a high specific capacity of 723.7 mAh·g^(-1) is maintained after 60 *** study provides a new strategy for a multifunctional sulfur host that can effectively alleviate the shuttle effect of liPSs and improve the utilization of sulfur active substances.

关键词： ZnO/ZnS heterostructure lithium-sulfur batteries density functional theory Anchor Conversion

A Loss-model-based Efficiency Optimization Control Method for Induction Traction System of High-speed Train under Emergency Self-propelled Mode

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CES Transactions on Electrical Machines and Systems 2024年第2期8卷 227-239页

作者： Yutong zhu Yaohua li Institute of Electrical Engineering Chinese Academy of Sciences Beijing 100190China University of Chinese Academy of Sciences Beijing 100190China China Academy of Railway Sciences Corporation limited(Beijing Zongheng Electromechanical Technology Co.Ltd Beijing 100081 China IEEE

Increasing attention has been paid to the efficiency improvement of the induction traction system of high-speed trains due to the high demand for energy saving. In emergency self-propelled mode, however, the dc-link voltage and the traction power of the motor are significantly reduced, resulting in decreased traction efficiency due to the low load and low speed operations. Aiming to tackle this problem, a novel efficiency improved control method is introduced to the emergency mode of high-speed train traction system in this paper. In the proposed method, a total loss model of induction motor considering the behaviors of both iron and copper loss is established. An improved iterative algorithm with decreased computational burden is then introduced, resulting in a fast solving of the optimal flux reference for loss minimization at each control period. In addition, considering the parameter variation problem due to the low load and low speed operations, a parameter estimation method is integrated to improve the controller's robustness. The effectiveness of the proposed method on efficiency improvement at low voltage and low load conditions is demonstrated by simulated and experimental results.

关键词： Efficiency optimization Induction motor Loss model control Motor drives Traction system

DNA vaccine prime and replicating vaccinia vaccine boost induce robust humoral and cellular immune responses against MERS-CoV in mice

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Virologica Sinica 2024年第3期39卷 490-500页

作者： Xiuli Shen Shuhui Wang Yanling Hao Yuyu Fu li Ren Dan li Wenqi Tang Jing li Ran Chen Meiling zhu Shuo Wang ying liu Yiming Shao National Key Laboratory of Intelligent Tracking and Forecasting for Infectious Diseases National Center for AIDS/STD Control and PreventionChinese Center for Disease Control and PreventionBeijing 102206China Changping Laboratory Yard 28Science Park RoadChangping DistrictBeijing 102206China State Key Laboratory for Diagnosis and Treatment of Infectious Diseases National Clinical Research Center for Infectious DiseasesCollaborative Innovation Center for Diagnosis and Treatment of Infectious DiseasesThe First Affiliated HospitalSchool of MedicineZhejiang UniversityHangzhou 310003China

As of december 2022,2603 laboratory-identified Middle East respiratory syndrome coronavirus(MERS-CoV)infections and 935 associated deaths,with a mortality rate of 36%,had been reported to the World Health Organization(WHO).However,there are still no vaccines for MERS-CoV,which makes the prevention and control of MERS-CoV *** this study,we generated two DNA vaccine candidates by integrating MERS-CoV Spike(S)gene into a replicating Vaccinia Tian Tan(VTT)*** to homologous immunization with either vaccine,mice immunized with DNA vaccine prime and VTT vaccine boost exhibited much stronger and durable humoral and cellular immune *** immunized mice produced robust binding antibodies and broad neutralizing antibodies against the EMC2012,England1 and KNIH strains of ***-Boost immunization also induced strong MERS-S specific T cells responses,with high memory and poly-functional(CD107a-IFN-γ-TNF-α)effector CD8t T *** conclusion,the research demonstrated that DNA-Prime/VTT-Boost strategy could elicit robust and balanced humoral and cellular immune responses against *** study not only provides a promising set of MERS-CoV vaccine candidates,but also proposes a heterologous sequential immunization strategy worthy of further development.

关键词： MERS-CoV VTT vaccine DNA vaccine Humoral and cellular immune responses Prime/boost strategy

Old Pinus massoniana forests benefit more from recent rapid warming in humid subtropical areas of central-southern China

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Journal of Forestry Research 2024年第5期35卷 155-170页

作者： Wenxin li liangjun zhu lianhua zhu Mengdan Jing Censhi Qian Yu zhu Paolo Cherubini College of life Science and Technology Central South University of Forestry and TechnologyChangsha 410004People’s Republic of China Swiss Federal Institute for Forest Snow and Landscape Research WSL8903 BirmensdorfSwitzerland department of Forest and Conservation Sciences Faculty of ForestryUniversity of British Columbia2004-2424 Main MallVancouverBC V6T 1Z4Canada

Trees progress through various growth stages,each marked by specific responses and adaptation strate-gies to environmental *** the importance of age-related growth responses on overall forest health and management policies,limited knowledge exists regarding age-related effects on dendroclimatic relationships in key subtropical tree *** this study,we employed a den-drochronological method to examine the impact of rapid warming on growth dynamics and climatic sensitivity of young(40–60 years)and old(100–180 years)Pinus mas-soniana forests across six sites in central-southern *** normalized log basal area increment of trees in both age groups increased significantly following rapid warming in *** in young forests further showed a distinct growth decline during a prolonged severe drought(2004–2013),whereas those in old forests maintained growth *** growth was more strongly influenced by temperature than by moisture,particularly in old *** tem-peratures strongly and positively impacted the growth of old trees but had a weaker effect on young *** forests had a significantly lower resistance to extreme drought but faster recovery compared to young ***“divergence problem”was more pronounced in younger forests due to their heightened sensitivity to warming-induced drought and heat *** ongoing warming,young forests also may initially experience a growth decline due to their heightened sensitivity to winter *** findings underscore the importance of considering age-dependent changes in forest/tree growth response to warming in subtropical forest man-agement,particularly in the context of achieving“Carbon Peak&Carbon Neutrality”goals in China.

关键词： Tree rings Pinus massoniana Age effects Drought resilience Subtropical forests Rapid warming

design Optimization of a Self-circulated Hydrogen Cooling System for a PM Wind Generator Based on Taguchi Method

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CES Transactions on Electrical Machines and Systems 2024年第2期8卷 170-176页

作者： Gaojia zhu Yunhao li Longnv li IEEE School of Electrical Engineering Tiangong UniversityTianjin 300387China

With the continuous improvement of permanent magnet(PM)wind generators'capacity and power density,the design of reasonable and efficient cooling structures has become a *** paper proposes a fully enclosed self-circulating hydrogen cooling structure for a originally forced-air-cooled direct-drive PM wind *** proposed hydrogen cooling system uses the rotor panel supports that hold the rotor core as the radial blades,and the hydrogen flow is driven by the rotating plates to flow through the axial and radial vents to realize the efficient cooling of the *** to the structural parameters of the cooling system,the Taguchi method is used to decouple the structural *** influence of the size of each cooling structure on the heat dissipation characteristic is analyzed,and the appropriate cooling structure scheme is determined.

关键词： Permanent magnet wind generator Hydrogen cooling Taguchi method Fluidic-thermal coupled fields

Electroacupuncture Promotes Functional Recovery after Facial Nerve Injury in Rats by Regulating Autophagy via GDNF and PI3K/m TOR Signaling Pathway

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Chinese Journal of Integrative Medicine 2024年第3期30卷 251-259页

作者： YAO Jun-peng FENG Xiu-mei WANG Lu li Yan-qiu zhu Zi-yue YAN Xiang-yun YANG Yu-qing li ying ZHANG Wei Acupuncture and Tuina School Chengdu University of Traditional Chinese MedicineChengdu611137China department of Rehabilitation Medicine Guanghan People’s HospitalGuanghanSichuan Province618399China Academic Affairs Office Chengdu University of Traditional Chinese MedicineChengdu611137China

Objective:To explore the mechanism of electroacupuncture(EA) in promoting recovery of the facial function with the involvement of autophagy,glial cell line-derived neurotrophic factor(GDNF),and phosphatidylinositol-3-kinase(PI3K)/mammalian target of rapamycin(mTOR) signaling ***:Seventy-two male Sprague-Dawley rats were randomly allocated into the control,sham-operated,facial nerve injury(FNI),EA,EA+3-methyladenine(3-MA),and EA+GDNF antagonist groups using a random number table,with 12 rats in each *** FNI rat model was established with facial nerve crushing *** intervention was conducted at Dicang(ST 4),Jiache(ST 6),Yifeng(SJ 17),and Hegu(li 4) acupoints for 2 *** Simone’s 10-Point Scale was utilized to monitor the recovery of facial *** histopathological evaluation of facial nerves was performed using hematoxylin-eosin(HE) *** levels of Beclin-1,light chain 3(LC3),and P62 were detected by immunohistochemistry(IHC),immunofluorescence,and reverse transcriptionpolymerase chain reaction,***,IHC was also used to detect the levels of GDNF,Rai,PI3K,and ***:The facial functional scores were significantly increased in the EA group than the FNI group(Plin sheaths,which were destroyed immediately after the injury,were recovered with EA *** expressions of Beclin-1 and LC3 were significantly elevated and the expression of P62 was markedly reduced in FNI rats(Pdel of *** may exert this neuroreparative effect through

关键词： electroacupuncture facial nerve injury autophagy glial cell line-derived neurotrophic factor phosphatidylinositol-3-kinase/mammalian target of rapamycin Chinese medicine

Assessments of Data-Driven deep Learning Models on One-Month Predictions of Pan-Arctic Sea Ice Thickness

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Advances in Atmospheric Sciences 2024年第7期41卷 1379-1390页

作者： Chentao SONG Jiang zhu Xichen li Institute of Atmospheric Physics Chinese Academy of SciencesBeijing 100029China University of Chinese Academy of Sciences Beijing 100049China

In recent years,deep learning methods have gradually been applied to prediction tasks related to Arctic sea ice concentration,but relatively little research has been conducted for larger spatial and temporal scales,mainly due to the limited time coverage of observations and reanalysis ***,deep learning predictions of sea ice thickness(SIT)have yet to receive ample *** this study,two data-driven deep learning(DL)models are built based on the ConvLSTM and fully convolutional U-net(FC-Unet)algorithms and trained using CMIP6 historical simulations for transfer learning and fine-tuned using reanalysis/*** models enable monthly predictions of Arctic SIT without considering the complex physical processes *** comprehensive assessments of prediction skills by season and region,the results suggest that using a broader set of CMIP6 data for transfer learning,as well as incorporating multiple climate variables as predictors,contribute to better prediction results,although both DL models can effectively predict the spatiotemporal features of SIT *** the predicted SIT anomalies of the FC-Unet model,the spatial correlations with reanalysis reach an average level of 89%over all months,while the temporal anomaly correlation coefficients are close to unity in most *** models also demonstrate robust performances in predicting SIT and SIE during extreme *** effectiveness and reliability of the proposed deep transfer learning models in predicting Arctic SIT can facilitate more accurate pan-Arctic predictions,aiding climate change research and real-time business applications.

关键词： Arctic sea ice thickness deep learning spatiotemporal sequence prediction transfer learning

Genomic variation,environmental adaptation,and feralization in ramie,an ancient fiber crop

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Plant Communications 2024年第8期5卷 189-207页

作者： Zeng-Yuan Wu Mark A.Chapman Jie liu Richard I.Milne ying Zhao Ya-Huang Luo Guang-Fu zhu Marc W.Cadotte Ming-Bao Luan Peng-Zhen Fan Alex K.Monro Zhi-Peng li Richard T.Corlett de-zhu li Germplasm Bank of Wild Species&Yunnan Key Laboratory of Crop Wild Relatives Omics Kunming Institute of BotanyChinese Academy of SciencesKunmingYunnan 650201China School of Biological Sciences University of SouthamptonSouthampton SO171BJUK CAS Key Laboratory for Plant Diversity and Biogeography of East Asia Kunming Institute of BotanyChinese Academy of SciencesKunmingYunnan 650201China Institute of Molecular Plant Sciences School of Biological SciencesUniversity of EdinburghEdinburgh EH93JHUK department of Biological Sciences University of Toronto-ScarboroughTorontoOntarioCanada Institute of Bast Fiber Crops Chinese Academy of Agricultural SciencesChangshaHunan 410205China Royal Botanic Gardens Kew RichmondSurrey TW93AEUK Center for Integrative Conservation and Yunnan Key Laboratory for the Conservation of Tropical Rainforests and Asian Elephants Xishuangbanna Tropical Botanical GardenChinese Academy of SciencesMenglunYunnan 666303China

Feralization is an important evolutionary process,but the mechanisms behind it remain poorly ***,we use the ancient fiber crop ramie(Boehmeria nivea(L.)Gaudich.)as a model to investigate genomic changes associated with both domestication and *** first produced a chromosome-scale de novo genome assembly of feral ramie and investigated structural variations between feral and domesticated ramie ***,we gathered 915 accessions from 23 countries,comprising cultivars,major landraces,feral populations,and the wild *** on whole-genome resequencing of these accessions,we constructed the most comprehensive ramie genomic variation map to ***,demographic,and admixture signal detection analyses indicated that feral ramie is of exoferal or exo-endo origin,i.e.,descended from hybridization between domesticated ramie and the wild progenitor or ancient *** ramie has higher genetic diversity than wild or domesticated ramie,and genomic regions affected by natural selection during feralization differ from those under selection during *** analyses showed that feral and domesticated ramie have similar ecological niches that differ substantially from the niche of the wild progenitor,and three environmental variables are associated with habitat-specific adaptation in feral *** findings advance our understanding of feralization,providing a scientific basis for the excavation of new crop germplasm resources and offering novel insights into the evolution of feralization in nature.

关键词： ramie fiber offering