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Characteristics of patients with Wilson disease in the United States: An insurance claims database study

World Journal of Hepatology 2024年第5期16卷 791-799页

作者： thomas daniel-r.bin Pradeep Kumar Bernard Benichou Jean-Philippe Combal Vivet Therapeutics Paris 75008France Clarivate Noida 201301Uttar PradeshIndia

BACKGrOUND Wilson disease(WD)is a progressive,potentially fatal degenerative disease affecting the liver and central nervous *** its low prevalence,collecting data on large cohorts of patients with WD is *** insur-ance claims databases provide powerful tools to collect retrospective data on large numbers of patients with rare *** To describe patients with WD in the United States,their treatment and clinical outcome,using a large insurance claims *** This retrospective,longitudinal study was performed in the Clarivate real-World Data Product *** patients with≥2 claims associated with an Interna-tional Classification of Diseases 10(ICD-10)diagnostic code for WD(E83.01)between 2016 and 2021 were included and followed until death or study *** were divided into two groups by whether or not they were documented to have received a specific treatment for *** manifestations,hospital-isations,liver transplantation and death were *** Overall,5376 patients with an ICD-10 diagnostic code for WD were *** mean age at inclusion was 41.2 years and 52.0%were men.A specific WD treatment was documented for 885 patients(15.1%),although the number of patients taking zinc salts may be underestimated due to over the counter *** inclusion,the mean age of patients with a documented treatment was 36.6±17.8 years vs 42.2±19.6 years in those without a documented *** follow-up,273 patients(5.1%)*** with the American general population,the standardised mortality ratio was *** proportion of patients with a documented WD-specific treatment who died during follow-up was 4.0%and the mean age at death 52.7 *** Patients treated for WD in the United States had an excess early mortality compared with the American *** findings indicate that there is a significant unmet need for effective treatment for WD in the United States.

关键词： Wilson disease Mortality Treatment Copper chelators Zinc acetate United States

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Characterization of the infectivity of an Indonesian Zika virus strain in mammalian cell lines

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Asian Pacific Journal of Tropical Biomedicine 2024年第5期14卷 215-224页

作者： Justus thomas Obiajulu Sievers Anom Bowolaksono r.Tedjo Sasmono Graduate School of Biology Department of BiologyFaculty of Mathematics and Natural SciencesUniversitas IndonesiaDepok 16424Indonesia Eijkman research Center for Molecular Biology National Research and Innovation AgencyCibinong 16911Indonesia Cellular and Molecular Mechanisms in Biological System research Group Department of BiologyFaculty of Mathematics and Natural SciencesUniversitas IndonesiaDepok 16424Indonesia

Objective:To characterize the infection patterns and growth characteristics of the Zika virus(ZIKV)strain JMB-185 from Indonesia in various mammalian cell ***:ZIKV was grown in human(A549,HEK293,HepG2,Huh7,Jurkat,and THP-1)and non-human mammalian(rAW264.7,Vero,and Vero76)cell *** replication kinetics were measured using plaque assay,while intra-and extracellular viral rNA concentrations were assessed using *** cytometry was used to quantify the infected cells and cell viability was measured using an MTT *** ability of ZIKV to infect cell lines was visualized using a fluorescence immunostaining ***:This ZIKV strain preferentially infected the lung,kidney,and liver cell lines A549,HEK293,Huh7,Vero,and Vero76,but not the immune cells Jurkat,rAW264.7,and *** contrast,the ZIKV showed no sign of infection in HepG2 cells,while maintaining viral titer over 3 days post-infection,with no infection recorded in immunostaining,no increase in viral rNA,and no indication of cell ***:The Indonesian ZIKV strain has a similar infection profile as other strains,except for its poor infectivity on HepG2 *** on the growth characteristics of Indonesia ZIKV will help expand our understanding of the biology of ZIKV which will be useful for various applications including antiviral discovery.

关键词： Zika virus Infectivity replication Cell lines In vitro

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Insights into carbon dioxide sequestration into coal seams through coupled gas flow-adsorption-deformation modelling

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Journal of rock Mechanics and Geotechnical Engineering 2024年第1期16卷 26-40页

作者： Hywel thomas Min Chen Geoenvironmental research Centre Cardiff School of EngineeringCardiff UniversityQueen’s BuildingsThe ParadeNewport RoadCardiffCF243AAUK Key Laboratory of Marine Geology Tongji UniversityShanghai200092China

Injecting carbon dioxide(CO_(2))into coal seams may unlock substantial carbon sequestration *** the coal acts like a carbon filter,it can preferentially absorb significant amounts of CO_(2).To explore this further,desorption of the adsorbed gas due to pressure drop is investigated in this paper,to achieve an improved understanding of the long-term fate of injected CO_(2) during post-injection *** paper presents a dual porosity model coupling gas flow,adsorption and geomechanics for studying coupled processes and effectiveness of CO_(2) sequestration in coals.A new adsorption?desorption model derived based on thermodynamics is incorporated,particularly,the desorption hysteresis is *** reliability of the proposed adsorption-desorption isotherm is examined via validation *** is indicated that occurrence of desorption hysteresis is attributed to the adsorption-induced pore *** injection ceases,the injected gas continues to propagate further from the injection well,while the pressure in the vicinity of the injection well experiences a significant *** the adsorbed gas near the well also decreases,this decrease is less compared to that in pressure because of desorption *** unceasing spread of CO_(2) and drops of pressure and adsorbed gas depend on the degree of desorption hysteresis and heterogeneity of coals,which should be considered when designing CO_(2) sequestration into coal seams.

关键词： CO_(2)geological storage Coal seam Adsorption Desorption hysteresis

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Distortions in focusing laser pulses due to spatio-temporal couplings:an analytic description

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High Power Laser Science and Engineering 2024年第3期12卷 20-40页

作者： Klaus Steiniger Fabia Dietrich daniel Albach Michael Bussmann Arie Irman Markus Loeser richard Pausch thomas Püschel roland Sauerbrey Susanne Schöbel Ulrich Schramm Mathias Siebold Karl Zeil Alexander Debus Helmholtz-Zentrum Dresden-rossendorf DresdenGermany CASUS-Center for Advanced Systems Understanding GörlitzGermany Technische Universität Dresden DresdenGermany

In ultra-short laser pulses,small changes in dispersion properties before the final focusing mirror can lead to severe pulse distortions around the focus and therefore to very different pulse properties at the point of laser±matter interaction,yielding unexpected interaction *** mapping between far-and near-field laser properties intricately depends on the spatial and angular dispersion properties as well as the focal *** a focused Gaussian laser pulse under the influence of angular,spatial and group-delay dispersion,we derive analytical expressions for its pulse-front tilt,duration and width from a fully analytic expression for its electric field in the time±space domain obtained with scalar diffraction *** expression is not only valid in and near the focus but also along the entire propagation distance from the focusing mirror to the *** relating angular,spatial and group-delay dispersion before focusing at an off-axis parabola,where they are well measurable,to the respective values in the pulse’s focus are obtained by a ray tracing ***,these formulas are used to show in example setups that the pulse-front tilts of lasers with small initial dispersion can become several tens of degrees larger in the vicinity of the focus while being small directly in the *** formulas derived here provide the analytical foundation for observations previously made in numerical *** numerically simulating Gaussian pulse propagation and measuring properties of the pulse at distances several rayleigh lengths off the focus,we verify the analytic expressions.

关键词： group-delay dispersion pulse-front tilt spatio-temporal couplings third-order dispersion ultra-short laser pulses

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CLASSIFICATIONS OF DUPIN HYPErSUrFACES IN LIE SPHErE GEOMETrY

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Acta Mathematica Scientia 2024年第1期44卷 1-36页

作者： thomas E.CECIL Department of Mathematics and Computer Science College of the Holy CrossWorcesterMA01610USA

This is a survey of local and global classification results concerning Dupin hypersurfaces in S^(n)(or r^(n))that have been obtained in the context of Lie sphere *** emphasis is on results that relate Dupin hypersurfaces to isoparametric hypersurfaces in *** with these classification results,many important concepts from Lie sphere geometry,such as curvature spheres,Lie curvatures,and Legendre lifts of submanifolds of S^(n)(or r^(n)),are described in *** paper also contains several important constructions of Dupin hypersurfaces with certain special properties.

关键词： Dupin hypersurfaces isoparametric hypersurfaces Lie sphere geometry Lie sphere transformations Lie curvatures

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Structural effects of reedbed grazing and its cessation on reed-nesting songbird densities

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Avian research 2024年第2期15卷 238-248页

作者： thomas Pagnon Clemence Pechinot Lea Sgro Jeremie Demay remi Jullian regis Gallais Brigitte Poulin Cyril Marmoex Theria Ecologie 6 avenue des martyrs05400VeynesFrance UMr 6249 Chrono-Environnement CNRSUniversite de Franche-Comte16 route de Gray25000BesanconFrance Conservatoire d’Espaces Naturels d’Occitanie 26 alle de Mycenes34000MontpellierFrance Office Français de la Biodiversite 12 cours Lumiere94300VincennesFrance Tour du Valat research Institute for the Conservation of Mediterranean Wetlands Le Sambuc13200ArlesFrance

reedbeds are crucial breeding habitats for vulnerable songbird *** of their protection status,these habitats may be threatened by organic matter accumulation,progressively leading to structural homoge-nization and habitat succession towards *** prevent excessive litter build-up with various in-terventions opening-up the reedbeds,such as grazing,which may be detrimental or suitable for some reed bird *** assessed the effects of extensive grazing by horses and its cessation in the medium and long terms on reedbed structure,and the consequences on reed-nesting songbird densities in Estagnol Nature reserve,a pro-tected wetland near the French Mediterranean *** compared reedbed structural features between grazed,newly ungrazed and old-ungrazed *** nine years,we censused four songbird species in spring and collected water level data in the same survey *** reduced reedbed extent,rejuvenated the vegetation with more short green stems on a thinner litter,and produced higher structural heterogeneity and discontinuity compared to long-lasting non *** ungrazed plot showed intermediate *** surveyed songbird total densities were similar among plots while species numbers and densities *** reedbed was more attractive to Moustached Warblers(Acrocephalus melanopogon)and Great reed Warblers(Acrocephalus arundi-naceus),likely due to the large edges and the high vegetation structural ***,Moustached Warblers were more negatively affected by higher water level in grazed reedbed,presumably because flooding prevents litter *** newly ungrazed reedbed was not preferred by any *** warbler species were found under low density in the old-ungrazed reedbed,where reed Buntings(Emberiza schoeniclus)were exclu-sively *** availability related to thick litter layer may explain this *** reed Warblers(Acrocephalus scirpaceus)were found everywhere in similar

关键词： Bird conservation Edge complexity Fragmentation Habitat management Horse grazing Phragmites australis reed passerine

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Comparative Analysis: Trays versus Packed Columns in Pressure-Swing Distillation for the Separation of Tetrahydrofuran, Water and Ethanol Azeotropic Mixture

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World Journal of Engineering and Technology 2024年第3期12卷 798-819页

作者： Samuel Oluwaseun Ogunrinde Tolulope daniel Adekoya thomas A. Orhadahwe Department of Mechanical Engineering Faculty of Engineering Ajayi Crowther University Oyo Nigeria AstraZeneca Boston Massachusetts USA

This paper delves into the comparative study of tray and packed column pressure swing distillation systems, focusing on the separation of a ternary mixture containing ethanol, tetrahydrofuran (THF), and water. The study particularly emphasizes the production of 99.5 w/w% tetrahydrofuran from the downstream product of 1,4-butanediol synthesis via diethyl maleate. Pro/II simulation software is utilized to explore various system configurations, including sieve trays, valve trays, and packed columns. Material and energy balances are performed to ascertain stream compositions and energy demands. The investigation encompasses the effects of column operating pressure on condenser and reboiler temperatures, as well as the implications of utility streams. A rigorous distillation model is employed to compare valve tray, sieve tray, and random packing (utilizing Norton Super Intalox) column designs by varying the number of trays, reflux ratio, and second distillation column pressure. Heat exchangers are integrated into the model, and their areas and utility flow rates are computed and integrated into the economic assessment. Economic analysis, guided by Net Present Value (NPV) calculations over a 20-year span, drives the selection of the most cost-effective design. results demonstrate that while all designs are energy-efficient, the packed column system emerges as the most economical choice, offering a comprehensive framework for the separation process. Furthermore, optimal design configurations and operating conditions for both tray and packed column systems are outlined, providing valuable insights for industrial applications.

关键词： Azetrope Tetrahydrofuran Ethanol Pressure-Swing Distillation Simulation

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Monogenic deficiency in murine intestinal Cdc42 leads to mucosal inflammation that induces crypt dysplasia

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Genes & Diseases 2024年第1期11卷 413-429页

作者： Dongsheng Zhang Wenjuan Tang Haitao Niu William Tse Hai-bin ruan Helmut Dolznig thomas Knosel Friedrich Karl-Heinz Madeleine Themanns Jiang Wang Mingquan Song Lee Denson Lukas Kenner richard Moriggl Yi Zheng Xiaonan Han Division of Hematology and Oncology Division of Cancer BiologyDepartment of MedicineMetroHealth Medical Center(MHMC)Case Western Reserve University(CWRU)School of MedicineClevelandOH 44109USA Cancer Genomics and Epigenomics Program Case Comprehensive Cancer CenterCase Western Reserve University(CWRU)ClevelandOH 44106USA Division of Gastroenterology Hepatology and NutritionCincinnati Children’s Hospital Medical Center(CCHMC)CincinnatiOH 45229USA Children’s Hospital of Fudan University Shanghai 201102China School of Medicine Jinan UniversityGuangzhouGuangdong 510632China Laboratory Animal Science(ILAS) Chinese Academy of Medical Science(CAMS)and Peking Union Medical College(PUMC)Beijing 100006China Department of Integrative Biology and Physiology University of Minnesota Medical SchoolMinneapolisMI 55455USA Institute of Medical Genetics Medical University of ViennaVienna 1040Austria Institute of Pathology Ludwig-Maximilians-University MunichMunich 80539Germany Institute of Biochemistry II University Hospital JenaJena 07743Germany Laboratory Animal Pathology University of Veterinary Medicine ViennaVienna 1210Austria Department of Pathology University of CincinnatiCincinnatiOH 45221USA Department of Gastroenterology The Affiliated Hospital of Qingdao UniversityQingdaoShandong 266005China Department of Pathology Medical University of ViennaVienna 1040Austria Ludwig Boltzmann Institute for Cancer research Vienna 1090Austria Medical University of Vienna Vienna 1040Austria Institute of Animal Breeding and Genetics University of Veterinary Medicine ViennaVienna 1210Austria Division of Experimental Hematology CCHMCCincinnatiOH 45229USA

CDC42 controls intestinal epithelial(IEC)stem cell(IESC)*** aberrant CDC42 initiates intestinal inflammation or neoplasia is *** utilized models of inflam-matory bowel diseases(IBD),colorectal cancer,aging,and IESC injury to determine the loss of intestinal Cdc42 upon inflammation and *** specimens were collected to determine the levels of CDC42 in IBD or colorectal ***42 floxed mice were crossed with Villin-Cre,Villin-CreErT2 and/or Lgr5-eGFP-IrES-CreErT2,or Bmi1-CreErT2 mice to generate Cdc42 deficient ***,colitis,aging,and intestinal organoid were used to evaluate CDC42 upon mucosal inflammation,IESC/progenitor regenerative capacity,and IEC *** studies revealed that increased CDC42 in colorectal cancer correlated with lower survival;in contrast,lower levels of CDC42 were found in the inflamed IBD *** Cdc42 depletion significantly reduced Lgr5+IEsCs,increased progenitors'hyperplasia,and induced mucosal inflammation,which led to crypt *** Cdc42 depletion markedly enhanced irra-diation-or chemical-induced *** or inhibition of Cdc42 reduced colonic Lgr5+IESC *** conclusion,depletion of Cdc42 reduces the IESC regeneration and IEC repair,leading to prolonged mucosal *** monogenic loss of Cdc42 in-duces mucosal inflammation,which could result in intestinal neoplasia in the context of aging.

关键词： Cell divisioncycle 42(CDC42) Colitis Colorectal cancer(CrC) Inflammatory bowel diseases(IBD) Intestinal epithelial cell(IEC) Intestinal epithelial stem cell(IESC) Irradiation

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Influence of water evaporation on elastohydrodynamic lubrication with water-containing polyalkylene glycols

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Friction 2024年第10期12卷 2370-2388页

作者： Stefan HOFMANN thomas LOHNEr Karsten STAHL Technical University of Munich School of Engineering and DesignDepartment of Mechanical EngineeringGear Research Center(FZG)Boltzmannstraße 1585748 Garching near MunichGermany

The reduction of frictional power losses in power transmitting gears takes a crucial role in the design of energy-and resource-efficient ***-containing lubricants like glycerol and polyalkylene glycols have shown great potential in achieving friction within the superlubricity regime with coefficients of friction lower than 0.01 under elastohydrodynamic ***,a bio-based production of the base stocks can lead to the development of green ***,one challenge associated with the application of water-containing lubricants to gearboxes is the evaporation of water and its impact on the lubricant *** this study,the influence of water evaporation on elastohydrodynamic friction and film thickness was investigated for three water-containing polyalkylene *** nominal water contents of 20 wt%and 40 wt%and two viscosities were *** results show that the friction increases continuously with higher evaporated water content,while the overall friction level remains low in nearly water-free states.A similar trend is observed for film thickness,where the strong increase in viscosity results in a notable increase in film ***,the sensitivity of friction and film thickness to water evaporation is low for small amounts of evaporated *** allows generous thresholds for permissible variations in water content.

关键词： friction superlubricity aqueous lubrication elastohydrodynamic lubrication(EHL) evaporation polyalkylene glycol

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Theory and classification of mass extinction causation

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National Science review 2024年第1期11卷 16-36页

作者： thomas J.Algeo Jun Shen State Key Laboratory of Biogeology and Environmental Geology China University of Geosciences—Wuhan State Key Laboratory of Geological Processes and Mineral resources China University of Geosciences—Wuhan Department of Geosciences University of Cincinnati

Theory regarding the causation of mass extinctions is in need of systematization, which is the focus of this contribution. Every mass extinction has both an ultimate cause, i.e. the trigger that leads to various climato-environmental changes, and one or more proximate cause(s), i.e. the specific climato-environmental changes that result in elevated biotic mortality. With regard to ultimate causes, strong cases can be made that bolide(i.e. meteor) impacts, large igneous province(LIP) eruptions and bioevolutionary events have each triggered one or more of the Phanerozoic Big Five mass extinctions, and that tectono-oceanic changes have triggered some second-order extinction events. Apart from bolide impacts, other astronomical triggers(*** flares, gamma bursts and supernova explosions) remain entirely in the realm of speculation. With regard to proximate mechanisms, most extinctions are related to either carbon-release or carbon-burial processes, the former being associated with climatic warming, ocean acidification, reduced marine productivity and lower carbonate δ13C values, and the latter with climatic cooling, increased marine productivity and higher carbonate δ13C values. Environmental parameters such as marine redox conditions and terrestrial weathering intensity do not show consistent relationships with carbon-cycle *** this context, mass extinction causation can be usefully classified using a matrix of ultimate and proximate factors. Among the Big Five mass extinctions, the end-Cretaceous biocrisis is an example of a bolide-triggered carbon-release event, the end-Permian and end-Triassic biocrises are examples of LIP-triggered carbon-release events, and the Late Ordovician and Late Devonian biocrises are examples of bioevolution-triggered carbon-burial events. Whereas the bolide-impact and LIP-eruption mechanisms appear to invariably cause carbon release, bioevolutionary triggers can result in variable carbon-cycle changes, e.g. carbon burial durin

关键词： biocrisis ultimate cause proximate cause bolide large igneous province bioevolutionary event

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