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Fabry Disease: Update, Focusing on Heart Disease by Multimodal imaging

World Journal of Cardiovascular Diseases 2024年第6期14卷 351-362页

作者： Adrian Espejel-Guzman Emily Rodríguez Valente Fernandez-Badillo Javier Serrano-Roman Aldo Cabello-Ganem Alexis Daniel Aparicio-Ortiz Alberto Ramon-Rios Mariali Palacios-cruz Nilda Espinola-Zavaleta Department of Nuclear Cardiology. National institute of Cardiology ignacio Chavez Mexico City Mexico ABC Medical Center IAP Department of Echocardiography Mexico City Mexico

Fabry disease (FD) is a rare X-linked lysosomal accumulation disorder caused by a deficiency in the enzyme alpha-galactosidase A (Gal A), resulting in excessive storage of glycosphingolipids, particularly globotriaosylceramide (Gb3). This leads to cellular dysfunction in various organs, with cardiovascular compromise being the major cause of morbidity and mortality. This study aimed to provide a comprehensive overview of FD focusing on its genetic, epidemiological, clinical, diagnostic, and therapeutic aspects. This study explored the genetic mutations associated with FD, its epidemiology, clinical phenotypes, cardiac manifestations, diagnostic approaches, and current treatment options. Background: FD is caused by mutations in GLA on the X chromosome, with over 1000 identified variants. Neonatal screening and specific studies have shown an increased incidence of FD. The clinical presentation varies between classic and late phenotypes, with cardiac involvement being a major concern, particularly in late-onset FD. Purpose: This study aimed to summarize the current knowledge on FD, emphasizing cardiac involvement, diagnostic modalities, and treatment options. Methods: A literature review of relevant studies on FD, including genetics, epidemiology, clinical presentation, diagnostic methods, and treatment options, was conducted. Results: Cardiac manifestations of FD included left ventricular hypertrophy (LVH), heart failure, arrhythmias, and sudden death. Diagnostic approaches such as electrocardiography, echocardiography, and cardiac magnetic resonance imaging play crucial roles in the early detection and monitoring of cardiac involvement. Enzyme replacement therapy (ERT) and emerging treatments have shown promise in managing FD, although challenges remain. conclusions: FD remains a challenging condition in cardiology, with under-diagnosis being a concern. Early detection and specific therapy are essential to improve patient outcomes. Echocardiography and cardiac MRi ar

关键词： Fabry Disease Hypertrophic Cardiomyopathy Echocardiography Cardiac Magnetic Resonance imaging Enzyme Replacement Therapy

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Preventing bad behavior in academia

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四川生理科学杂志 2024年第7期46卷 1615-1615页

作者： Rayane da cruz Albino Faculty of Pharmacy Federal University of Rio de Janeiro Rio de Janeiro RJ Brazil. Department of Host-Microbe interactions St. Jude Children’s Research Hospital Memphis TN USA. School of the Environment The University of Queensland Whiterock QLD Australia. KU Leuven Ghent Belgium. Laboratory for computational Social Systems Indraprastha Institute of Information Technology Delhi New Delhi India. University Medical college Shantou Guangdong China. Department of Ecoscience Aarhus Universitet Aarhus Denmark. institute of Environment and Sustainable Development Banaras Hindu University Varanasi Uttar Pradesh India. Department of Medical Parasitology Faculty of Medicine and Health Sciences Sana’a University Sana’a Yemen and Tropical Disease Research Center Faculty of Medicine and Health Sciences University of Science and Technology Sana’a Yemen. Department of Medicine University of Montreal Montreal QC Canada. Electricity infrastructure and Buildings Division Pacific Northwest National Laboratory Portland OR USA. Department of Biological Sciences University of New Brunswick Saint John Saint John NB Canada. Department of Biomedical Engineering The University of Melbourne Melbourne VIC Australia. Vagelos Molecular Life Sciences Program University of Pennsylvania Philadelphia PA USA. Doctoral Program in Neurobiology and Behavior Columbia University New York NY USA. Department of Genetics and Genomic Sciences Icahn School of Medicine at Mount Sinai New York NY USA. Department of Engineering University of Cambridge Cambridge Cambridgeshire UK. Department of Geography Liverpool John Moores University Liverpool UK. Department of Biology Stanford University Stanford CA USA. Department of Psychology University of Nicosia Nicosia Cyprus. Department of Global Ecology Carnegie Institution for Science Stanford CA USA. Research and Support Division Stellenbosch University Cape Town South Africa.

We gave young scientists this prompt:Describe one change to scientific policy or culture that would substantially decrease incidents of scientific misconduct or other unethical behavior.

关键词： behavior. prompt culture

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淮北煤田中高阶煤储层物性表征及深部有利区预测研究

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非常规油气 2024年

作者：侯海海 Elizabeth cruz Pérez 陈泓圳胡博何倩黄乡琴辽宁工程技术大学矿业学院

为定量表征淮北煤田中高阶煤储层物性特征，采用工业分析、显微煤岩组分定量、低温氮吸附、高压压汞、核磁共振等实验手段，对淮北煤田7个煤样的物性特征进行了研究。结果表明，淮北煤田煤显微组分以镜质组为主，最大镜质组反射率变化... 详细信息

为定量表征淮北煤田中高阶煤储层物性特征，采用工业分析、显微煤岩组分定量、低温氮吸附、高压压汞、核磁共振等实验手段，对淮北煤田7个煤样的物性特征进行了研究。结果表明，淮北煤田煤显微组分以镜质组为主，最大镜质组反射率变化较大，在0.7%-2.5%之间，可能受燕山期郯庐和太行山岩浆活动的共同影响。该煤质具有中-高灰分、低-极低硫含量、低磷、中高热值的特点。从低阶煤到中阶煤，由于脱水作用较强，煤基质收缩，吸附孔隙和渗流孔隙体积减小，裂隙体积增大；从中阶煤到高阶煤，吸附孔具有明显的优势，表明其对甲烷的吸附能力增强。预测淮北煤田宿县矿区的南坪深部、桃园祁南深部、彭桥深部、朱仙庄北部，临涣矿区的任楼赵集深部、许疃深部，濉萧矿区的萧西深部为该区煤层气勘探的较有利区，

关键词：淮北煤田煤储层物性孔裂隙结构煤层气有利区优选

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University corporate Governance in the Literature From 2019 to 2024

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Journalism and Mass communication 2024年第3期14卷 179-188页

作者： María del Rosario Molina González María Teresa Gaxiola Sánchez María de Jesús Camargo Pacheco Marco Antonio Velderrain Rodríguez Rafael Campoy Mendoza cruz García Lirios Universidad de Sonora NavojoaMexico

Anti-pandemic policies impacted the governance of Higher Education institutions(HEis)through confinement and distancing *** an impact was observed in the identity,reputation,and image of the HEi in the face of the health *** objective of this work was to establish an empirical contrast between the theoretical structure consulted regarding the evaluations of summaries published from 2019 to 2024 by judges.A documentary,cross-sectional,and retrospective study was carried out with a sample of sources indexed in international *** results demonstrate that image and age regulate the impact of identity and income on *** relation to the state of the art,it is recommended to extend the study to sociocultural,demographic,economic,and educational variables in order to anticipate the transition from university governance to corporate governance.

关键词： coViD governance model nodes neural networks

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Study of tree shrew biology and models: A booming and prosperous field for biomedical research

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Zoological Research 2024年第4期45卷 877-909页

作者： Yong-Gang Yao Li Lu Rong-Jun Ni Rui Bi Ceshi Chen Jia-Qi Chen Eberhard Fuchs Marina Gorbatyuk Hao Lei Hongli Li Chunyu Liu Long-Bao Lv Kyoko Tsukiyama-Kohara Michinori Kohara Claudia Perez-cruz Gregor Rainer Bao-Ci Shan Fang Shen An-Zhou Tang Jing Wang Wei Xia Xueshan Xia Ling Xu Dandan Yu Feng Zhang Ping Zheng Yong-Tang Zheng Jumin Zhou Jiang-Ning Zhou Key Laboratory of Genetic Evolution and Animal Models Yunnan Key Laboratory of Animal Models and Human Disease MechanismsKunming Institute of ZoologyChinese Academy of SciencesKunmingYunnan 650204China National Research Facility for Phenotypic&Genetic Analysis of Model Animals(Primate Facility) and National Resource Center for Non-Human PrimatesKunming Institute of ZoologyChinese Academy of SciencesKunmingYunnan 650107China KiZ-CUHK Joint Laboratory of Bioresources and Molecular Research in common Diseases Kunming Institute of ZoologyChinese Academy of SciencesKunmingYunnan 650204China Kunming college of Life Science University of Chinese Academy of SciencesKunmingYunnan 650204China Mental Health Center and Psychiatric Laboratory West China HospitalSichuan UniversityChengduSichuan 610041China Sichuan Clinical Medical Research Center for Mental Disorders ChengduSichuan 610044China German Primate Center Leibniz Institute of Primate ResearchGöttingen 37077Germany Department of Optometry and Vision Science School of OptometryUniversity of Alabama at BirminghamBirminghamAL 35294USA State Key Laboratory of Magnetic Resonance and Atomic and Molecular Physics Innovation Academy for Precision Measurement Science and TechnologyChinese Academy of SciencesWuhanHubei 430071China Wuhan National Laboratory for Optoelectronics Huazhong University of Science and TechnologyWuhanHubei 430074China Soong Ching Ling institute of Maternity and Child Health International Peace Maternity and Child Health HospitalShanghai Jiao Tong University School of MedicineShanghai 200030China Transboundary Animal Diseases Center Joint Faculty of Veterinary MedicineKagoshima UniversityKagoshima-cityKagoshima 890-8580Japan Department of Microbiology and Cell Biology Tokyo Metropolitan Institute of Medical ScienceTokyo 156-8506Japan Pharmacology Department CinvestavMexico City 07360Mexico Department of Medicine University of FribourgFribourg CH-1700Switzerland Beijing Engineering Research Center of Radiog

The tree shrew(Tupaia belangeri)has long been proposed as a suitable alternative to non-human primates(NHPs)in biomedical and laboratory research due to its close evolutionary relationship with *** recent years,significant advances have facilitated tree shrew studies,including the determination of the tree shrew genome,genetic manipulation using spermatogonial stem cells,viral vector-mediated gene delivery,and mapping of the tree shrew brain ***,the limited availability of tree shrews globally remains a substantial challenge in the ***,determining the key questions best answered using tree shrews constitutes another *** shrew models have historically been used to study hepatitis B virus(HBV)and hepatitis C virus(HCV)infection,myopia,and psychosocial stress-induced depression,with more recent studies focusing on developing animal models for infectious and neurodegenerative *** these efforts,the impact of tree shrew models has not yet matched that of rodent or NHP models in biomedical *** review summarizes the prominent advancements in tree shrew research and reflects on the key biological questions addressed using this *** emphasize that intensive dedication and robust international collaboration are essential for achieving breakthroughs in tree shrew *** use of tree shrews as a unique resource is expected to gain considerable attention with the application of advanced techniques and the development of viable animal models,meeting the increasing demands of life science and biomedical research.

关键词： Tree shrew Animal model Neurodegenerative diseases infectious diseases Neuroscience Phenome

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Venopulmonary ECMO improved Hypoxemia and Supported the Right Ventricle in a Patient with Decompensated Eisenmenger Syndrome

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World Journal of Cardiovascular Surgery 2024年第1期14卷 7-19页

作者： Daniel Manzur-Sandoval Gian Manuel Jiménez-Rodríguez Edgar García-cruz Ramón Espinosa-Soto Erika Yamali Ramirez-Marcano Yessenia Máyory Téllez-López José Luis Elizalde-Silva Gustavo Rojas-velasco Cardiovascular Critical Care Unit Ignacio Ch&#225vez National Institute of Cardiology Mexico City Mexico Adult congenital Heart Disease Unit Ignacio Ch&#225vez National Institute of Cardiology Mexico City Mexico Cardiology Department Ignacio Ch&#225vez National Institute of Cardiology Mexico City Mexico

Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure refractory to standard therapies. Adult patients with congenital heart disease are an increasingly recognized and growing population and include various groups, such as undiagnosed cases in childhood and palliated and/or corrected cases, which require subsequent care because of residual lesions, cardiac arrest/insufficiency, and arrhythmias, among other conditions. in addition, these patients are prone to developing pathologies that are typical of adulthood with a generally increased risk of morbidity and mortality because of their low reserves and organic damage associated with the underlying heart disease, which makes them candidates for ECMO. These patients represent an additional challenge in this therapy because malformations and the presence of a shunt can generally affect the usual cannulation methods and hemodynamic and oximetry monitoring. Thus, the configuration decision must be made on a case-by-case basis. Here, we present a cannulation method, venopulmonary artery ECMO, which provides hemodynamic and respiratory support, and may be ideal for patients with shunts and/or right ventricular dysfunction. To our knowledge, this is the first report of this configuration in patients with congenital heart diseases.

关键词： Eisenmenger Syndrome Venopulmonary Artery ECMO Refractory Hypoxemia Right Ventricular Dysfunction

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insights on Chemical and Natech Risk Management in Japan and South Korea:A Review of Current Practices

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international Journal of Disaster Risk Science 2022年第3期13卷 359-371页

作者： Hyejeong Park Ana Maria cruz Disaster Prevention Research institute Kyoto UniversityKyoto611-0011Japan

A growing number of natural hazard-triggered technological accidents(Natech)has been reported by several researchers,and this trend is expected to continue due to climate *** a result,some governments have initiated direct efforts to manage Natech risks,particularly in the United States and ***,two surveys conducted by the Organisation for Economic co-operation and Development(OECD)in 2009 and 2017 found that there was a lack of proper risk management and risk governance for Natech among OECD member states,including Japan and South *** study aimed to identify relevant regulations and practical considerations for chemical and Natech risk management from government perspectives in Japan and South *** article provides a review of the current state of risk management,emergency response,and risk communication on chemical and Natech risk management in the two countries,and concludes with a discussion of some of the issues that require improvement of the current chemical risk *** practices for chemical risk management in Japan and South Korea point to the possibility of improvements in dealing with the Natech *** practical lessons will be valuable for improving the capacity for dealing with challenges in chemical and Natech risk management.

关键词： Chemical accidents Government perspectives Japan Natech Risk governance Risk management South Korea

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Model of Mediating Determinants of Time Differences in iNDYCAR

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Journalism and Mass communication 2024年第4期14卷 232-240页

作者： Wilfrido isidro Aldana Balderas Héctor Daniel Molina Ruiz Enrique Martínez Muñoz Eleazar Villegas González Tirso Javier Hernández Gracia Arturo Sánchez Sánchez cruz García Lirios Alfredo Barrera Escobar University of México HuehuetocaMèxico University of Hidalgo PachucaMèxico University of Tlaxcala Mèxico University of CDMX Mexico University of FV Mexico

The indyCar series distinguishes itself by providing the same design and operation of the single-seater to its *** difference in times is then attributable to the skills of the drivers,but considering the data from the races could test this *** objective of this work was to establish a trajectory model to predict race times.A cross-sectional,correlational,and explanatory work was carried out with a sample of 18,474 records in the period from 2020 to 2023 of the indyCar *** results show that the time span predicts the time *** relation to the studies of acceptance of the technology,the adjustment of this to human capacities to explain the time differences in the series of racing cars is discussed.

关键词： time differences ranking differences ranking race time laps completed

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Global stability for multi-species Lotka-Volterra cooperative systems： One hyper-connected mutualistic-species

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international Journal of Biomathematics 2015年第3期8卷 215-223页

作者： cruz Vargas-De-Leon Unidad de Medicina Experimental Hospital General de Mexico"Dr. Eduardo Liceaga" DF 06726 Mexico

We propose two models of one hyper-connected mutualistic-species described by delay differential equations of Lotka-Volterra type. An hyper-connected model comprises a central species interacting with a number of peripheral species around it, that is to say, one animal species （pollinators or dispersers） that interacts with several plant species （flowering plants or fruit trees）, or several animal species that interact with one plant species. We derive a necessary and sufficient condition for the global asymptotic stability of the unique coexisting steady state of hyper-connected systems by means of novel Lyapunov functionals.

关键词： Lotka Volterra cooperative system discrete delays distributed delays Lyapunov functional global stability.

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Utilizing a novel fiber optic technology to capture the axial responses of fully grouted rock bolts

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Journal of Rock Mechanics and Geotechnical Engineering 2018年第2期10卷 222-235页

作者： Nicholas Vlachopoulos Daniel cruz Bradley Forbes Department of Civil Engineering Royal Military College of Canada Department of Geological Sciences and Geological Engineering Queen's UniversityKingston

Rock bolts are one of the primary support systems utilized in underground excavations within the civil and mining engineering industries. Rock bolts support the weakened rock mass adjacent to the opening of an excavation by fastening to the more stable, undisturbed formations further from the excavation. The overall response of such a support element has been determined under varying loading conditions in the laboratory and in situ experiments in the past four decades; however, due to the limitations with conventional monitoring methods of capturing strain, there still exists a gap in knowledge associated with an understanding of the geomechanical responses of rock bolts at the microscale. in this paper, we try to address this current gap in scientific knowledge by utilizing a newly developed distributed optical strain sensing(DOS) technology that provides an exceptional spatial resolution of 0.65 mm to capture the strain along the rock bolt. This DOS technology utilizes Rayleigh optical frequency domain reflectometry(ROFDR) which provides unprecedented insight into various mechanisms associated with axially loaded rebar specimens of different embedment lengths, grouting materials, borehole annulus conditions, and borehole diameters. The embedment length of the specimens was found to be the factor that significantly affected the loading of the rebar. The critical embedment length for the fully grouted rock bolts(FGRBs) was systematically determined to be430 mm. The results herein highlight the effects of the variation of these individual parameters on the geomechanical responses FGRBs.

关键词： Fiber optic technology Fully grouted rock bolts Load transfer Stress distribution

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