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Clinical characteristics and management of coexistent cardiomyopathy in patients with bicuspid aortic valve

Journal of Geriatric Cardiology 2023年第3期20卷 205-213页

作者： Di ZHANG yi-ting lu Ze-Min ZHOU Yu-Xiao HU Xin-Chang LIU yi QU Ya-Xin LIU Xian-Liang ZHOU Department of Cardiology Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Emergency and Critical Care Center Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina

BACKGROUND Bicuspid aortic valve(BAV)is the most common congenital heart ***,the prevalence,clinical characteristics,and current management of BAV associated with inherited cardiomyopathy,including hypertrophic cardiomy-opathy(HCM),dilated cardiomyopathy(DCM),and left ventricular noncompaction(LVNC)have not been well *** Consecutive patients diagnosed with BAV at a large tertiary cardiovascular referral center between 2009 and 2018 were retrospectively assessed for HCM,DCM,and LVNC based on clinical and echocardiographic *** with coexist-ent conditions were investigated *** Of 3533 patients with BAV screened,57(1.6%)had concomitant *** was combined with HCM in 30 of these patients,with DCM in 19,and with LVNC in ***-six patients(80.7%)were male,and the mean age at first dia-gnosis was 47 years for BAV with HCM,49 years for BAV with DCM,and 35 years for BAV with *** failure and aortic valve dysfunction were common in these patients,and the prevalence of coexisting aortopathy was 43.3%,26.3%and 25.0%,re-spectively,for BAV with HCM,DCM and *** the index hospitalization,24 of the 57 patients(42.1%)underwent sur-gery,16(28%)underwent aortic valve and/or aortic surgery,and 16 of the 30 patients with HCM had a Morrow *** were no deaths or other major adverse cardiovascular *** The prevalence of inherited cardiomyopathy was higher in our patients with BAV than in the general *** and heart failure were common,with almost half of patients requiring surgery at diagnosis.

关键词： patients cardiomyopathy aortic

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A novel missense mutation in obscurin gene in a Chinese consanguineous family with left ventricular noncompaction

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Journal of Geriatric Cardiology 2022年第7期19卷 531-538页

作者： Xue-Qi DONG Pei-Pei QIN Di ZHANG Qiong-Yu ZHANG yi QU Lin ZHAO yi-ting lu Yu-Xiao HU Chun-Xue YANG Xin-Chang LIU Ya-Xin LIU Xian-Liang ZHOU Department of Cardiology Fuwai HospitalNational Center for Cardiovascular DiseaseChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Department of Obstetrics and Gynecology Peking Union Medical College HospitalBeijingChina

BACKGROUND Left ventricular noncompaction(LVNC) is an increasingly recognised cardiomyopathy of which a significant percentage are genetic in origin. The purpose of the present study was to identify potential pathogenic mutation leading to disease in a Chinese LVNC *** A 3-generation family affected by LVNC was recruited. Clinical assessments were performed on available family members, with clinical examination, ECG, echocardiography and cardiac MRI. The proband(Ⅰ-2), the proband’s daughter(Ⅱ-1, affected) and mother(Ⅲ-1, unaffected) were selected for WGS. Sanger sequencing were performed in all of the 4 surviving family *** Combined whole genome sequencing with linkage analysis identified a novel missense mutation in the giant protein obscurin(OBSCN NM_001098623, c.C19063T), as the only plausible disease-causing variant that segregates with disease among the four surviving individuals, with interrogation of the entire genome excluding other potential causes. This c.C19063T missense mutation resulted in p.R6355W in the encoded OBSCN protein. It affected a highly conserved residue in the C terminus of the obscurin-B-like isoform between the PH and STKc domains, which was predicted to affect the function of the protein by different bioinformatics *** Here we present clinical and genetic evidence implicating the novel R6355W missense mutation in obscurin as the cause of familial LVNC. This expands the spectrum of obscurin’s roles in cardiomyopathies. It furthermore highlights that rare obscurin missense variants, currently often ignored or left uninterpreted, should be considered to be relevant for cardiomyopathies and can be identified by the approach presented here. This study also provided new insights into the molecular basis of OBSCN mutation positive LVNC.

关键词： ventricular mother linkage

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Identifying the dynamic behaviors in complete reconstruction of Co-based complex precatalysts during electrocatalytic oxygen evolution

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Journal of Energy Chemistry 2025年第1期100卷 226-233页

作者： Jingfang Zhang Danyang Wu Linke Cai Youluan lu Fanpeng Cheng Lijuan Shi Qun yi Yao Liu yi Huang Key Laboratory of Green Chemical Engineering Process of Ministry of Education Engineering Research Center of Phosphorus Resources Development and Utilization of Ministry of Education Hubei Key Laboratory of Novel Reactor and Green Chemical Technology School of Chemical Engineering & Pharmacy Wuhan Institute of Technology Key Laboratory of Pesticide & Chemical Biology of Ministry of Education Engineering Research Center of Photoenergy Utilization for Pollution Control and Carbon Reduction of Ministry of Education College of Chemistry Central China Normal University College of Materials Science and Engineering Fuzhou University

Transition metal-based nanomaterials have emerged as promising electrocatalysts for oxygen evolution reaction（OER）. Considerable research efforts have shown that self-reconstruction occurs on these nanomaterials under operating conditions of OER process. However, most of them undergo incomplete reconstruction with limited thickness of reconstruction layer, leading to low component utilization and arduous exploration of real catalytic mechanism. Herein, we identify the dynamic behaviors in complete reconstruction of Co-based complexes during OER. The hollow phytic acid（PA） cross-linked CoFe-based complex nanoboxes with porous nanowalls are designed because of their good electrolyte penetration and mass transport ability, in favor of the fast and complete reconstruction. A series of experiment characterizations demonstrate that the reconstruction process includes the fast substitution of PA by OH-to form Co（Fe）(OH)xand subsequent potential-driven oxidation to Co（Fe）OOH. The obtained CoFeOOH delivers a low overpotential of 290 mV at a current density of 10 mA cm-2and a long-term *** experiment results together with theory calculations reveal that the Fe incorporation can result in the electron rearrangement of reconstructed CoFeOOH and optimization of their electronic structure,accounting for the enhanced OER activity. The work provides new insights into complete reconstruction of metal-based complexes during OER and offers guidelines for rational design of high-performance electrocatalysts.

关键词： Complete reconstruction Co-based complex Precatalysts Oxygen evolution reaction

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Atomically precise nickel-sulfur clusters for efficient electrochemical 2,5-hydroxymethylfurfural oxidation

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Nano Research 2025年第1期18卷 140-148页

作者： Fen Wang lu-Yu Liu Sha-Sha Cui ting-ting Li Xiao-Meng Yang Zhijuan Liu Yanyong Wang Henan Key Laboratory of Crystalline Molecular Functional Materials Henan International Joint Laboratory of Tumor Theranostical Cluster MaterialsGreen Catalysis Centerand College of ChemistryZhengzhou UniversityZhengzhou 450001China State Key Laboratory of Chem/Bio-sensing and Chemometrics College of Chemistry and Chemical EngineeringHunan UniversityChangsha 410082China

Nickel sulfide exhibits excellent catalytic activity in the electrochemical 2,5-hydroxymethylfurfural oxidation reaction(HMFOR).However,due to the polydispersity of nanoparticles,it is difficult to establish a clear structure-activity relationship at the atomic *** this work,we have successfully synthesized atomically precise Ni_(6)(PET)_(12)and Ni_(4)(PET)_(8)clusters(PET:2-phenylethanethiol)for ***^(2+)and S_(2)-with atomic ratio of 1:2 was mainly existed in Ni_(6)(PET)_(12)and Ni_(4)(PET)_(8)to form Ni-S *** electrochemical test results have suggested both Ni_(6)(PET)_(12)and Ni_(4)(PET)_(8)displayed outstanding electrocatalytic ability for *** Ni_(6)(PET)_(12)exhibited better electrocatalytic ability than Ni_(4)(PET)_(8)with higher current density,lower overpotential and faster reaction *** superior electrochemical ability of Ni_(6)(PET)_(12)may be due to the enhanced adsorption towards HMF molecule with strong interaction towards hydroxyl group and furan ***,it found that the Ni^(2+)species in Ni_(6)(PET)_(12)could rapidly oxidized into Ni^(3+)species,which could spontaneously capture electron and proton from HMF for *** theoretical calculation demonstrated that the Ni_(6)(PET)_(12)process lower free energy barrier than Ni_(4)(PET)_(8)to display excellent electrocatalytic *** work is of great significance for designing efficient electrocatalysts for HMFOR.

关键词： atomically precise nickel nanoclusters 2 5-hydroxymethylfurfural electro-oxidation Ni3+species

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南泥湾不同干扰湿地蝶类多样性

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生态学杂志 2017年第10期36卷 2840-2846页

作者：谭灿王亭贻王璐樊德苗马娟刘长海延安大学生命科学学院陕西延安716000 陕西省区域生物资源保育与利用工程技术研究中心陕西延安716000

2015—2016年,在南泥湾不同干扰强度4块湿地南泥湾村(YGS)、后九龙泉(TGS)、孟家沟(THS)、大南沟(WTS)共采集329号标本,分属鳞翅目5科27属36种,粉蝶科在4块样地均为优势科,菜粉蝶为地区优势种。区系分析发现:个体数量上广布种(W)占55.6%... 详细信息

2015—2016年,在南泥湾不同干扰强度4块湿地南泥湾村(YGS)、后九龙泉(TGS)、孟家沟(THS)、大南沟(WTS)共采集329号标本,分属鳞翅目5科27属36种,粉蝶科在4块样地均为优势科,菜粉蝶为地区优势种。区系分析发现:个体数量上广布种(W)占55.6%,古北种(P)和东洋种(O)分别占39.2%和5.2%,在物种数量上古北种(P)占50%,广布种(W)和东洋种(O)分别占36.1%和13.9%。在4块样地中,属数、种数、个体数趋势相同,WTS的属数、种数、个体数均最高,分别为23、27、132,其他依次是THS、TGS、YGS;物种丰富度(R)、多样性指数(H)、均匀度指数(J)趋势相同,WTS的R、H、J均最高,依次为5.3248、0.4393、0.1333,其次是THS、TGS、YGS;YGS优势度指数最高,为0.3265,其次是TGS、THS、WTS。不同干扰强度4块样地随干扰强度增加生境复杂程度逐渐降低,依次是WTS、THS、TGS、YGS。结果显示,从蝶类资源状况来看,南泥湾湿地生态演替仍未达到顶级,蝶类资源不丰富。

关键词：蝶类多样性干扰区系南泥湾湿地

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Understanding the local structure and thermophysical behavior of Mg-La liquid alloys via machine learning potential

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International Journal of Minerals,Metallurgy and Materials 2025年第2期32卷 439-449页

作者： Jia Zhao Taixi Feng Guimin lu National Engineering Research Center for Integrated Utilization of Salt Lake Resource East China University of Science and TechnologyShanghai 200237China Joint International Laboratory for Potassium and Lithium Strategic Resources East China University of Science and TechnologyShanghai 200237China

The local structure and thermophysical behavior of Mg-La liquid alloys were in-depth understood using deep potential molecular dynamic(DPMD) simulation driven via machine learning to promote the development of Mg-La alloys. The robustness of the trained deep potential(DP) model was thoroughly evaluated through several aspects, including root-mean-square errors(RMSEs), energy and force data, and structural information comparison results;the results indicate the carefully trained DP model is reliable. The component and temperature dependence of the local structure in the Mg-La liquid alloy was analyzed. The effect of Mg content in the system on the first coordination shell of the atomic pairs is the same as that of temperature. The pre-peak demonstrated in the structure factor indicates the presence of a medium-range ordered structure in the Mg-La liquid alloy, which is particularly pronounced in the 80at% Mg system and disappears at elevated temperatures. The density, self-diffusion coefficient, and shear viscosity for the Mg-La liquid alloy were predicted via DPMD simulation, the evolution patterns with Mg content and temperature were subsequently discussed, and a database was established accordingly. Finally, the mixing enthalpy and elemental activity of the Mg-La liquid alloy at 1200 K were reliably evaluated,which provides new guidance for related studies.

关键词： magnesium-lanthanum liquid alloys local structure macroscopic properties thermodynamic behavior deep potential mo-lecular dynamic simulation

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Lipomatous ependymoma with ZFTA:RELA fusion-positive:A case report

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World Journal of Clinical Cases 2025年第1期13卷 48-55页

作者： Xiao-Yu Zhao Juan-Han Yu yi-Hua Wang yi-Xin Liu lu Xu Lin Fu Ning yi China Medical University The First Hospital of China Medical UniversityShenyang 110001Liaoning ProvinceChina Department of Pathology The First Hospital of China Medical UniversityShenyang 110001Liaoning ProvinceChina Anshan Cancer Hospital Anshan 114000Liaoning ProvinceChina

BACKGROUND Ependymoma with lipomatous differentiation is a rare type of *** ZFTA fusion-positive supratentorial ependymoma is a novel tumor type in the 2021 World Health Organization classification of central nervous system *** fusion-positive lipomatous ependymoma has not been reported to *** SUMMARY We reported a case of a 15-year-old Chinese male who had a sudden convulsion lasting approximately six *** resonance imaging showed a round cystic shadow of approximately 1.9 cm×1.5 cm×1.9 cm under the right parieto-occipital *** examination showed characteristic perivascular pseudorosettes and adipose differentiation in the ***-chemical staining showed that the tumor cells were negative for cytokeratin,NeuN,Syn and p53,but positive for GFAP,vimentin and S-100 ***-ficant punctate intracytoplasmic EMA immunoreactivity was *** level of Ki-67 was about 5%.Genetic analysis revealed ZFTA:RELA fusion.A cranio-tomy with total excision of the tumor was *** follow-up time was 36 months,no evidence of disease recurrence was found in magnetic resonance *** Based on these findings,the patient was diagnosed as a ependymoma with ZFTA fusion and lipomatous *** case report provides information on the microscopic morphological features of ependymoma with ZFTA fusion and lipomatous differentiation,which can help pathologists to make a definitive diagnosis of this tumor.

关键词： Ependymoma Lipomatous Molecular classification Supratentorial ZFTA:RELA fusion Case report

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基于Fe3O4@AuNPs/MWCNTs-COOH纳米免疫传感器快速测定水产品的呋喃它酮代谢产物

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食品科学 2025年第2期46卷 222-230页

作者：李亭何欢歌鲁航李书国河北科技大学食品与生物学院河北石家庄050018

采用磁性四氧化三铁(Fe3O4)、纳米金(gold nanoparticles,AuNPs)、羧基化多壁碳纳米管(carboxylated multi-walled carbon nanotubes,MWCNTs-COOH)复合纳米材料固定化呋喃它酮代谢产物3-氨基-5-多酚甲基-2-恶唑烷酮(3-amino-5-methylmorpholino-2-oxazolidinone,AMOZ)抗体制备磁性纳米免疫传感器,建立一种快速检测水产品中呋喃它酮代谢产物的方法。利用紫外-可见光谱和红外光谱技术对制备的纳米Fe3O4和纳米复合材料Fe3O4@AuNPs的结构进行分析,利用扫描电子显微镜和透射电子显微镜对纳米材料的微观形貌进行表征;运用循环伏安法、电化学交流阻抗法对传感器的电化学性能进行表征,以[Fe(CN)6]^(3-/4-)为探针,用微分脉冲伏安法对实验条件进行优化,优化结果如下:Fe3O4@AuNPs和MWCNTs-COOH体积比为1∶2,电解质溶液pH值为7.0,抗体质量浓度为80μg/mL,孵育温度为37℃,孵育时间为30 min。在此条件下,该免疫响应电流与lg C[AMOZ]在1.0×10^(-10)~8.0×10^(-7)mol/L范围内具有良好的线性关系,检出限为2.4×10^(-11)mol/L,在鲫鱼样品实际检测中,加标回收率为92.78%~102.61%,与高效液相色谱-串联质谱法检测结果基本一致,但该法具有前处理简便、检测速度快的优势,可用于实际水产品中呋喃它酮代谢产物AMOZ的快速检测。

关键词：食品安全呋喃它酮代谢产物纳米免疫传感器水产品快速检测

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Protein-based functional hydrogel improves cutaneous nerve repair and diabetic wound healing

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Nano Research 2025年第1期18卷 586-596页

作者： Hanzhi lu Mingrui Cui yi Wang Xinran Du Xinyi Zhou Yuhan Fang Xiaoyan Gao ying Peng Jianyong Zhu Guang Yang Fulun Li Department of Dermatology Yueyang Hospital of Integrated Traditional Chinese and Western MedicineShanghai University of Traditional Chinese MedicineShanghai 200437China Shanghai Engineering Research Center of Nano-Biomaterials and Regenerative Medicine College of Biological Science and Medical EngineeringDonghua UniversityShanghai 201620China Clinical Laboratory Medicine Center Yueyang Hospital of Integrated Traditional Chinese and Western MedicineShanghai University of Traditional Chinese MedicineShanghai 200437China

Cutaneous nerve regeneration in diabetic wounds remains a challenge for current clinical ***,a natural protein-based functional hydrogel is developed using keratin as the sole matrix,and protocatechuic aldehyde(PA)and zinc ions as the building blocks through dual-dynamic crosslinking reactions of thiol-aldehyde addition and catecholzinc ions *** unique structural design endows the hydrogel with excellent injectability,skin adhesion,self-healing and antibacterial ability,and *** addition,this hydrogel acts as a drug carrier for the loading and sustained release of phellopterin(PP),a natural compound extracted from traditional Chinese medicine Angelica *** findings demonstrate that the PP-loaded hydrogel promotes cutaneous nerve regeneration,angiogenesis and tissue remodeling in the full-thickness wounds in both db/db and streptozotocin-induced C57BL/6 diabetic mice,notably by the synergistic effect of zinc ions and PP,showing a great potential in diabetic wound therapy.

关键词： functional hydrogel cutaneous nerve regeneration dynamic crosslinking diabetic wounds

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领导倾听内涵及其多层次效应

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心理科学进展 2025年第1期33卷 25-41页

作者：刘庚韩翼卢俊阳江西农业大学经济管理学院南昌330045 中南财经政法大学工商管理学院武汉430073 江西师范大学历史文化与旅游学院南昌330022

面对错综复杂和快速变化的商业环境,组织要想做出快速响应,领导者的有效倾听显得非常重要。通过文献综述发现,现有研究尚未对领导倾听的概念形成统一界定,且理论视角和研究层次较为单一,这不利于我们深入理解领导倾听的内涵及其多层次... 详细信息

面对错综复杂和快速变化的商业环境,组织要想做出快速响应,领导者的有效倾听显得非常重要。通过文献综述发现,现有研究尚未对领导倾听的概念形成统一界定,且理论视角和研究层次较为单一,这不利于我们深入理解领导倾听的内涵及其多层次效应。事实上,作为一种领导行为,领导倾听不仅会对领导者自身产生影响,而且会给团队及其下属带来影响。因此,本研究基于二元互动情境,从沟通信息和支持员工两方面对领导倾听的内涵进行探索性研究,并从领导力效能的评价对象(领导者、团队、员工)出发,结合多层次、多理论、多研究方法的全景研究范式逐步揭示领导倾听对不同对象的不同效应、黑箱机制及边界条件。本研究不仅开拓了倾听研究的新思路和新视角,而且为企业增强领导倾听的积极效能提供了管理启示。

关键词：领导倾听领导力效能领导工作绩效团队追随行为员工建言

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