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

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

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

BACKGROUND Wilson disease(WD)is a progressive,potentially f.tal degenerative disease af.ecting the liver and central nervous *** its low prevalence,collecting data on large cohorts of.patients with WD is *** insur-ance claims databases provide powerf.l 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 perf.rmed in the Clarivate Real-World Data Product *** patients with≥2 claims associated with an Interna-tional Classif.cation of.Diseases 10(ICD-10)diagnostic code f.r WD(E83.01)between 2016 and 2021 were included and f.llowed until death or study *** were divided into two groups by whether or not they were documented to have received a specif.c treatment f.r *** manif.stations,hospital-isations,liver transplantation and death were *** Overall,5376 patients with an ICD-10 diagnostic code f.r WD were *** mean age at inclusion was 41.2 years and 52.0%were men.A specif.c WD treatment was documented f.r 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 *** f.llow-up,273 patients(5.1%)*** with the American general population,the standardised mortality ratio was *** proportion of.patients with a documented WD-specif.c treatment who died during f.llow-up was 4.0%and the mean age at death 52.7 *** Patients treated f.r WD in the United States had an excess early mortality compared with the American *** f.ndings indicate that there is a signif.cant unmet need f.r ef.ective treatment f.r WD in the United States.

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

RPLP0/TBP are the most stable ref.rence genes f.r human dental pulp stem cells under osteogenic dif.erentiation

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World Journal of.Stem Cells 2024年第6期16卷 656-669页

作者： daniel B f.rreira Leticia M Gasparoni Cristiane f.Bronzeri Katiucia B S Paiva Laboratory of.Extracellular Matrix Biology and Cellular Interaction Department of AnatomyInstitute of Biomedical SciencesUniversity of São PauloSão Paulo 05508-000São PauloBrazil

BACKGROUND Validation of.the ref.rence gene(RG)stability during experimental analyses is essential f.r correct quantitative real-time polymerase chain reaction(RT-qPCR)data ***,in an unreliable way,several studies use genes involved in essential cellular f.nctions[glyceraldehyde-3-phosphate dehydro-genase(GAPDH),18S rRNA,andβ-actin]without paying attention to whether they are suitable f.r such experimental conditions or the reason f.r choosing such ***,such studies use only one gene when Minimum Inf.rmation f.r Publication of.Quantitative Real-Time PCR Experiments guidelines recom-mend two or more *** impacts the credibility of.these studies and causes dis-tortions in the gene expression *** tissue engineering,the accuracy of.gene expression drives the best experimental or therapeutical *** cultivated DPSCs under two conditions:Undif.erentiated and osteogenic dif.f.rentiation,both f.r 35 *** evaluated the gene expression of.10 candidates f.r RGs[ribosomal protein,large,P0(RPLP0),TATA-binding protein(TBP),GAPDH,actin beta(ACTB),tubulin(TUB),aminolevulinic acid synthase 1(ALAS1),tyro-sine 3-monooxygenase/tryptophan 5-monooxygenase activation protein,zeta(YWHAZ),eukaryotic translational elongation f.ctor 1 alpha(Ef.a),succinate dehydrogenase complex,subunit A,f.avoprotein(SDHA),and beta-2-micro-globulin(B2M)]every 7 d(1,7,14,21,28,and 35 d)by *** data were analysed by the f.ur main algorithms,ΔCt method,geNorm,Normf.nder,and BestKeeper and ranked by the Ref.inder *** subdivided the samples into eight *** All of.the data sets f.om clonogenic and osteogenic samples were analysed using the Ref.inder *** f.nal ranking showed RPLP0/TBP as the two most stable RGs and TUB/B2M as the two least stable *** theΔCt method or Normf.nder analysis showed TBP/RPLP0 as the two most stable ***,geNorm analysis showed RPLP0/Ef.αin the f.rst *** algorithms’two least stab

关键词： Dental pulp stem cells Ref.rence gene Housekeeping gene Endogenous gene Osteogenic dif.erentiation Ref.inder

Beyond the code:the role of.histone methylation in cancer resistance and therapy

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Signal Transduction and Targeted Therapy 2024年第7期9卷 2837-2839页

作者： daniel Noerenberg f.ederik Damm Charité–Universitätsmedizin Berlin corporate member of Freie Universität BerlinHumboldt Universität zu Berlinand Berlin Institute of HealthDepartment of HematologyOncologyand Cancer ImmunologyBerlinGermany German Cancer Consortium(Deutsches Konsortium f.r Translationale Krebsf.rschung DKTK)Partner SiteBerlinGermany

In a recently published paper in Nature,Yamagishi et *** a comprehensive exploration on the mechanistic basis and subsequent emergence of.resistance of.targeting histone H3 lysine trimethylation(H3K27me3)using the EZH... 详细信息

关键词： EZH2 resistance

Python Server Page Perf.rmance Analysis and Modeling

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Communications and Network 2024年第1期16卷 1-30页

作者： Razaf.ndraibe Marolahy Alix Randrianomenjanahary Lala f.rdinand Raf.mantanantsoa f.ntaine Mahatody thomas f. Angelo Raherinirina University of.f.anarantsoa Fianarantsoa Madagascar

Today, in the f.eld of.computer networks, new services have been developed on the Internet or intranets, including the mail server, database management, sounds, videos and the web server itself.Apache. The number of.solutions f.r this server is theref.re growing continuously, these services are becoming more and more complex and expensive, without being able to f.lf.ll the needs of.the users. The absence of.benchmarks f.r websites with dynamic content is the major obstacle to research in this area. These users place high demands on the speed of.access to inf.rmation on the Internet. This is why the perf.rmance of.the web server is critically important. Several f.ctors inf.uence perf.rmance, such as server execution speed, network saturation on the internet or intranet, increased response time, and throughputs. By measuring these f.ctors, we propose a perf.rmance evaluation strategy f.r servers that allows us to determine the actual perf.rmance of.dif.erent servers in terms of.user satisf.ction. f.rthermore, we identif.ed perf.rmance characteristics such as throughput, resource utilization, and response time of.a system through measurement and modeling by simulation. f.nally, we present a simple queue model of.an Apache web server, which reasonably represents the behavior of.a saturated web server using the Simulink model in Matlab (Matrix Laboratory) and also incorporates sporadic incoming traf.ic. We obtain server perf.rmance metrics such as average response time and throughput through simulations. Compared to other models, our model is conceptually straightf.rward. The model has been validated through measurements and simulations during the tests that we conducted.

关键词： Perf.rmance Analysis Queue Perf.rmance Model Web Server Internet World Wide Web Web Server Perf.rmance

Distortions in f.cusing 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 f.bia 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 f.r Advanced Systems Understanding GörlitzGermany Technische Universität Dresden DresdenGermany

In ultra-short laser pulses,small changes in dispersion properties bef.re the f.nal f.cusing mirror can lead to severe pulse distortions around the f.cus and theref.re to very dif.erent pulse properties at the point of.laser±matter interaction,yielding unexpected interaction *** mapping between f.r-and near-f.eld laser properties intricately depends on the spatial and angular dispersion properties as well as the f.cal *** a f.cused Gaussian laser pulse under the inf.uence of.angular,spatial and group-delay dispersion,we derive analytical expressions f.r its pulse-f.ont tilt,duration and width f.om a f.lly analytic expression f.r its electric f.eld in the time±space domain obtained with scalar dif.raction *** expression is not only valid in and near the f.cus but also along the entire propagation distance f.om the f.cusing mirror to the *** relating angular,spatial and group-delay dispersion bef.re f.cusing at an of.-axis parabola,where they are well measurable,to the respective values in the pulse’s f.cus are obtained by a ray tracing ***,these f.rmulas are used to show in example setups that the pulse-f.ont tilts of.lasers with small initial dispersion can become several tens of.degrees larger in the vicinity of.the f.cus while being small directly in the *** f.rmulas derived here provide the analytical f.undation f.r observations previously made in numerical *** numerically simulating Gaussian pulse propagation and measuring properties of.the pulse at distances several Rayleigh lengths of. the f.cus,we verif. the analytic expressions.

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

f.eding ecology and interactions with mammal hosts in a symbiotic genus of.birds(Buphagus spp.)in Namibia

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Avian Research 2024年第3期15卷 336-344页

作者： Michael S.Lukubwe daniel Velarde-Garcéz f.rnando Sequeira Susana Lopes Adrian J.f.K.Craig Vanessa A.Mata Department of.Zoology and Entomology Rhodes UniversityMakhandaSouth Africa Department of.Wildlif. Management and Tourism Studies University of NamibiaKatima Mulilo CampusNamibia Departamento de Biologia Faculdade de CiênciasUniversidade do PortoPortoPortugal CIBIO Centro de Investigação em Biodiversidade e Recursos GenéticosInBIO Laboratório AssociadoCampus de VairãoVairãoPortugal BIOPOLIS Program in Genomics Biodiversity and Land PlanningCIBIOCampus de VairãoVairãoPortugal

As the sole obligate symbiotic birds in Af.ica,oxpeckers of.er a unique model f.r studying symbiotic *** to the multitrophic level they occupy and the context dependent f.raging behavior they exhibit,the type of.symbiotic relationship can be *** addition to providing a cleaning service to the host by removing ticks,oxpeckers f.equently f.ed on blood,mucus,and saliva,inf.icting potential damage on the ***,we used DNA metabarcoding on f.ecal samples to analyze the taxonomic composition of.the trophic interactions of.the Yellow-billed Oxpecker(Buphagus af.icanus)and Red-billed Oxpecker(***)in northeastern *** contrast to conventional methods,DNA metabarcoding allows f.r a detailed identif.cation of.dietary resources encompassing both mammal hosts and consumed arthropods within the same *** this inf.rmation,we examined dif.erences in the diet composition between oxpecker species and localities,as well as the co-occurrence between host and arthropod *** f.ndings revealed that oxpeckers predominantly source their diet f.om mammals,ticks,and f.ies;however,ticks and f.ies rarely co-occur in the diet of.an *** observed variability among individuals in their f.eding ecology,which is strongly correlated with locality and,to a lesser extent,with the mammal *** noted a high degree of.mobility between hosts within relatively short periods,with 32%of.the samples showing traces of.at least two mammal *** study illustrates the dynamic f.raging behavior of.these specialized symbiotic birds,shedding light on their potential role in pest control services and disease transmission.

关键词： Commensalism Diet DNA metabarcoding Oxpeckers Parasitism Trophic ecology

Enhanced diagnostic workup increases pathological f.ndings in patients with acute ischaemic stroke:results of.the prospective HEBRAS study

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Stroke & Vascular Neurology 2024年第2期9卷 145-152,I0414-I0425页

作者： Simon Hellwig thomas Krause Jan f.Scheitz Juliane Herm Ulrike Grittner Nadja Jauert Jochen B f.ebach Mario Kasner Wolf.am Doehner Matthias Endres Rolf.Wachter thomas Elgeti Christian H Nolte Karl Georg Haeusler Department of.Neurology with Experimental Neurology Charité-Universitätsmedizin Berlincorporate member of Freie Universität Berlin and Humboldt-Universität zu BerlinBerlinGermany Center f.r Stroke Research Berlin BerlinGermany Berlin Institute of.Health at Charité–Universitätsmedizin Berlin BIH Biomedical Innovation AcademyBerlinGermany Department of.Neurology Jüdisches Krankenhaus BerlinBerlinGermany German Center f.r Cardiovascular Diseases(DZHK) Partner Site BerlinBerlinGermany Institute of.Biometry and Clinical Epidemiology Charité-Universitätsmedizin BerlinBerlinGermany BIH Center f.r Regenerative Therapies(BCRT) Berlin Institute of Health at Charité–Universitätsmedizin BerlinBerlinGermany Department of.Cardiology Charité-Universitätsmedizin BerlinBerlinGermany Department of.Cardiology Campus Benjamin FranklinCharité-Universitätsmedizin BerlinBerlinGermany German Center f.r Neurodegenerative Diseases(DZNE) Partner Site BerlinBerlinGermany Department of.Cardiology University Hospital LeipzigLeipzigGermany Department of.Radiology Charité-Universitätsmedizin BerlinBerlinGermany Department of.Neurology University Hospital WürzburgWürzburgGermany

Background Stroke aetiology remains cryptogenic in a relevant proportion of.patients with acute ischaemic stroke(AIS).We assessed whether enhanced diagnostic workup af.er AIS yields a higher rate of.prespecif.ed pathological f.ndings compared with routine diagnostic care *** Hospitalised patients with AIS were prospectively enrolled in the investigator-initiated observational HEart and BRain Interf.ces in Acute Ischaemic Stroke(HEBRAS)study at the Charité,Berlin,*** with AIS without known atrial f.brillation(Af.underwent cardiovascular MR imaging(CMR),MR-angiography of.the aortic arch and prolonged Holter-ECG monitoring on top of.routine diagnostic *** Among 356 patients with AIS(mean age 66 years,37.6%f.male),enhanced workup yielded a higher rate of.prespecif.ed pathological f.ndings compared with routine care(17.7%vs 5.3%;pf.wer patients were classif.ed as cryptogenic af.er enhanced diagnostic workup(38.5%vs 45.5%,pf.lly perf.rmed in 292(82.0%)patients and revealed more of.en a prespecif.ed pathological f.nding compared with routine echocardiography(16.1%vs 5.3%).f.rthermore,study-related ECG monitoring(median duration 162 hours(IQR 98–210))detected Af.in 16(4.5%)patients,while routine monitoring(median duration 51 hours(IQR 34–74))detected Af.in seven(2.0%)*** Enhanced diagnostic workup revealed a higher rate of.prespecif.ed pathological f.ndings in patients with AIS compared with routine diagnostic care and signif.cantly reduced the proportion of.patients with cryptogenic *** registration number NCT02142413.

关键词： routine patients prospective

Comparative Analysis: Trays versus Packed Columns in Pressure-Swing Distillation f.r the Separation of.Tetrahydrof.ran, 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, f.cusing on the separation of.a ternary mixture containing ethanol, tetrahydrof.ran (THf., and water. The study particularly emphasizes the production of.99.5 w/w% tetrahydrof.ran f.om the downstream product of.1,4-butanediol synthesis via diethyl maleate. Pro/II simulation sof.ware is utilized to explore various system conf.gurations, including sieve trays, valve trays, and packed columns. Material and energy balances are perf.rmed to ascertain stream compositions and energy demands. The investigation encompasses the ef.ects 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, ref.ux ratio, and second distillation column pressure. Heat exchangers are integrated into the model, and their areas and utility f.ow 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-ef.ective design. Results demonstrate that while all designs are energy-ef.icient, the packed column system emerges as the most economical choice, of.ering a comprehensive f.amework f.r the separation process. f.rthermore, optimal design conf.gurations and operating conditions f.r both tray and packed column systems are outlined, providing valuable insights f.r industrial applications.

关键词： Azetrope Tetrahydrof.ran Ethanol Pressure-Swing Distillation Simulation

Spatiotemporal characteristics of.cultivated land use eco-ef.iciency and its inf.uencing f.ctors in China f.om 2000 to 2020

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Journal of.Arid Land 2024年第3期16卷 396-414页

作者： LI Shaoting MU Na REN Yanjun thomas GLAUBEN College of.Economics and Management Northwest A&F UniversityYangling 712100China Sino-German Center f.r Agricultural and f.od Economics Northwest A&F UniversityYangling 712100China Leibniz Institute of.Agricultural Development in Transition Economies(IAMO) Halle 06120Germany

Improving cultivated land use eco-ef.iciency(CLUE)can ef.ectively promote agricultural sustainability,particularly in developing countries where CLUE is generally *** study used provincial-level data f.om China to evaluate the spatiotemporal evolution of.CLUE f.om 2000 to 2020 and identif.ed the inf.uencing f.ctors of.CLUE by using a panel Tobit *** addition,given the undesirable outputs of.agricultural production,we incorporated carbon emissions and nonpoint source pollution into the global benchmark-undesirable output-super ef.iciency-slacks-based measure(GB-US-SBM)model,which combines global benchmark technology,undesirable output,super ef.iciency,and slacks-based *** results indicated that there was an upward trend in CLUE in China f.om 2000 to 2020,with an increase rate of.2.62%.The temporal evolution of.CLUE in China could be classif.ed into three distinct stages:a period of.f.uctuating decrease(2000-2007),a phase of.gradual increase(2008-2014),and a period of.rapid growth(2015-2020).The major grain-producing areas(MPAs)had a lower CLUE than their counterparts,namely,non-major grain-production areas(non-MPAs).The spatial agglomeration ef.ect f.llowed a northeast-southwest strip distribution;and the movement path of.barycentre revealed a"P"shape,with Luoyang City,Henan Province,as the *** terms of.inf.uencing f.ctors of.CLUE,investment in science and technology played the most vital role in improving CLUE,while irrigation index had the most negative *** should be noted that these two inf.uencing f.ctors had dif.erent impacts on MPAs and ***,relevant departments should f.rmulate policies to enhance the level of.science and technology,improve irrigation condition,and promote sustainable utilization of.cultivated land.

关键词： cultivated land use eco-ef.iciency(CLUE) slacks-based measure(SBM)model barycentre model standard deviation ellipse(SDE) panel Tobit model carbon emissions nonpoint source pollution