检索结果-南通市图书馆

Rathbun syndrome(hypophosphatasia)due to the heterozygous variant c.297+5G>A in alkaline phosphatase with unusual phenotype

在线全文

学校读者我要写书评

暂无评论

Chinese Medical Journal 2022年第3期135卷 377-378页

作者： Josef Finsterer Claudia stöllberger Department of Neurology Klinik LandstrasseMesserli InstituteViennaAustria nd Medical Department with Cardiology and Intensive Care Medicine Klinik LandstrasseViennaAustria

To the Editor:Hypophosphatasia(HPP)is a musculoskeletal inborn error-of-metabolism caused by variants in alkaline phosphatase(ALPL)with reduced activity of the serum tissue-non-specific alkaline phosphatase,[1]which is predominantly expressed in brain,muscle,bones,liver,and kidneys.[2]Accordingly,HPP is a multi-system disorder affecting bones and more rarely extra-osseus organs.[2]Osseus features of HPP include decreased bone quality,osteoid accumulations,reduced bone-mineralization,increased incidence of fractures,premature closure of sutures,bone deformities,dwarfism,Bechterew disease,early loss of teeth,intracranial hypertension,and prolonged bone healing.[1]Extra-osseous features of HPP include seizures,[3]myopathy,[2]hepatopathy,and renal *** responds favorably to enzyme replacement therapy with asfotase-α.[1]

关键词： metabolism healing organs

Spectrally tunable high-power Yb:fiber chirped-pulse amplifier

在线全文

学校读者我要写书评

暂无评论

Photonics Research 2022年第10期10卷 2309-2316页

作者： VALENTINA SHUMAKOVA VITO F.PECILE JAKOB FELLINGER MICHAEL LESKOWSCHEK P.E.COLLIN ALDIA ALINE S.MAYER LUKAS W.PERNER SARPER SALMAN MINGQI FAN PRANNAY BALLA stÉphane SCHILT CHRIstOPH M.HEYL INGMAR HARTL GIL PORAT OLIVER H.HECKL Christian Doppler Laboratory for Mid-IR Spectroscopy and Semiconductor Optics Faculty Center for Nano Structure ResearchFaculty of PhysicsUniversity of ViennaA-1090 ViennaAustria Photonics Institute TU WienA-1040 ViennaAustria Vienna Doctoral School in Physics University of ViennaA-1090 ViennaAustria Deutsches Elektronen-Synchrotron DESY 22607 HamburgGermany Laboratoire Temps-Fréquence Universitéde NeuchâtelCH-2000 NeuchâtelSwitzerland GSI Helmholtzzentrum für Schwerionenforschung GmbH 64291 DarmstadtGermany Helmholtz-Institute Jena 07743 JenaGermany Department of Electrical and Computer Engineering University of AlbertaEdmontonAlberta T6G 1H9Canada Department of Physics University of AlbertaEdmontonAlberta T6G 2E1Canada

Tailoring the properties of the driving laser to the need of applications often requires compromises among laser stability,high peak and average power levels,pulse duration,and spectral *** instance,spectroscopy with optical frequency combs in the extreme/visible ultraviolet spectral region requires a high peak power of the near-IR driving laser,and therefore high average power,pulse duration of a few tens of fs,and maximal available spectral ***,the parametric conversion efficiency is higher for pulses with a duration in the 100-fs range due to temporal walk-off and coating *** we suggest an approach to adjust the spectral characteristics of high-power chirped-pulse amplification(CPA)to the requirements of different nonlinear frequency converters while preserving the low-phase-noise(PN)properties of the *** achieve spectral tunability,we installed a mechanical spectral shaper in a free-space section of the stretcher of an in-house-developed ytterbium-fiber-based CPA *** CPA system delivers 100 W of average power at a repetition rate of 132.4 *** gaining control over the spectral properties,we preserve the relative-intensity-noise and PN properties of the *** high-power CPA can easily be adjusted to deliver either a spectrum ideal for mid-IR light generation(full width at half maximum of∼11 nm,compressed pulse duration of 230 fs)or a spectrum ideal for highly nonlinear processes such as high-harmonic generation(−10 dB level of>50 nm,transform-limited pulse duration of∼65 fs).

关键词： pulse power fiber

Quantitative structure-Activity Relationship study of a Benzimidazole-Derived Series Inhibiting Mycobacterium tuberculosis H37Rv

在线全文

学校读者我要写书评

暂无评论

Computational Chemistry 2022年第2期10卷 71-96页

作者： Georges stéphane Dembélé Mamadou Guy-Richard Koné Fandia Konate Doh Soro Nahossé Ziao Laboratoire de Thermodynamique et de Physico-Chimie du Milieu UFR SFA Université Nangui Abrogoua Abidjan C&#244 te-d’Ivoire Groupe Ivoirien de Recherches en Modélisation des Maladies (GIR2M) Université Nangui Abrogoua Abidjan C&#244 te-d’Ivoire

This work was carried out on a series of twenty-two (22) benzimidazole derivatives with inhibitory activities against Mycobacterium tuberculosis H37Rv by applying the Quantitative structure-Activity Relationship (QSAR) method. The molecules were optimized at the level DFT/B3LYP/6-31 + G (d, p), to obtain the molecular descriptors. We used three statistical learning tools namely, the linear multiple regression (LMR) method, the nonlinear regression (NLMR) and the artificial neural network (ANN) method. These methods allowed us to obtain three (3) quantitative models from the quantum descriptors that are, chemical potential (μ), polarizability (α), bond length l (C = N), and lipophilicity. These models showed good statistical performance. Among these, the ANN has a significantly better predictive ability R2 = 0.9995;RMSE = 0.0149;F = 31879.0548. The external validation tests verify all the criteria of Tropsha et al. and Roy et al. Also, the internal validation tests show that the model has a very satisfactory internal predictive character and can be considered as robust. Moreover, the applicability range of this model determined from the levers shows that a prediction of the pMIC of the new benzimidazole derivatives is acceptable when its lever value is lower than 1.

关键词： Mycobacterium tuberculosis H37Rv Benzimidazole Derivatives QSAR ANN Applicability Domain

Petrography of alluvial sands as a past and present environmental indicator:Case of the Loire River(France)

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

International Journal of Sediment Research 2013年第3期28卷 285-303页

作者： Jean-Jacques MACAIRE Isabelle GAY-OVEJERO Michel BACCHI Constantin COCIRTA Luc PATRYL stéphane RODRIGUES University Franqois-Rabelais of Tours University Francois-Rabelais of Tours SARL RIVE 4. Commissariat a l'Energie Atomique-CEA DAMDIF

Four sample sets of the Upper and Middle Loire river sands were analyzed in order to study the impact of natural and anthropogenic factors on their petrographic composition in space(on an 800 km stretch) and *** was determined by modal analysis of three sand-size fractions using a polarizing optical microscope and calculated for each sample("standard sand" = Sst).The watershed is composed mainly of endogenic(Massif Central) and sedimentary(southern Parisian Basin) rocks.B-set sands collected in channels for different water flows in 1996 show that Sst compositions vary by only 5%.Present-day sands in the Upper Loire and Middle Loire have very high petrographic immaturity comparing to others worldwide fluvial sands,although bio-climatic conditions favor sand maturation by source-rock weathering in the *** shows the strong impact of the Massif Central on sediment yield due to relief rejuvenation as a consequence of the formation of the Alps during the *** sands stored during the Weichselian and the Holocene in the Middle Loire floodplain,although partly weathered since their deposition,show higher inputs from the endogenic rocks of the Massif Central than present-day *** can be explained by Weichselian periglacial conditions and the development of crop farming since the Neolithic,which favored mechanical erosion,particularly in the Massif Central which is characterized by a cold,humid climate and steep *** upstream-downstream change in the composition of presently deposited sand is low in the diked *** shows however that basalt and some heavy mineral grains are vulnerable to abrasion during transport and indicates a marked sediment yield from ancient sediment stored in the *** is in line with the high incision of the river bed over the last 150 years partly due to dam construction and aggregate mining.

关键词： Fluvial sand Sand petrography Sediment source Tectonics impact Human impact Loire river

Internal Validity Index for Fuzzy Clustering Based on Relative Uncertainty

在线全文

同方期刊数据库

学校读者我要写书评

暂无评论

Computers, Materials & Continua 2022年第8期72卷 2909-2926页

作者： Refik Tanju Sirmen Burak Berk Üstündag Graduate School of Science Engineering&Technology Istanbul Technical UniversityIstanbul34469Turkey Faculty of Computer&Informatics Istanbul Technical UniversityIstanbul34469Turkey

Unsupervised clustering and clustering validity are used as essential instruments of data *** clustering being realized under uncertainty,validity indices do not deliver any quantitative evaluation of the uncertainties in the suggested ***,validity measures may be biased towards the underlying clustering ***,neglecting a confidence requirement may result in *** the absence of an error estimate or a confidence parameter,probable clustering errors are forwarded to the later stages of the ***,having an uncertainty margin of the projected labeling can be very fruitful for many applications such as machine ***,the validity issue was approached through estimation of the uncertainty and a novel low complexity index proposed for fuzzy *** involves only uni-dimensional membership weights,regardless of the data dimension,stipulates no specific distribution,and is independent of the underlying similarity *** tests and comparisons returned that it can reliably estimate the optimum number of partitions under different data distributions,besides behaving more robust to over ***,in the comparative correlation analysis between true clustering error rates and some known internal validity indices,the suggested index exhibited the highest strong *** relationship has been also proven stable through additional statistical acceptance *** the provided relative uncertainty measure can be used as a probable error estimate in the clustering as ***,it is the only method known that can exclusively identify data points in dubiety and is adjustable according to the required confidence level.

关键词： Machine learning data science clustering validity fuzzy clustering uncertainty intelligent systems data analytics

Emotions and performance in rugby

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Journal of Sport and Health Science 2019年第6期8卷 595-600页

作者： Mickael Campo stéphane Champely Andrew MLane Elisabeth Rosnet Claude Ferrand Benoit Louvet SPMS(EA 4180) UBFCDijon 21078France Laboratory for Vulnerabilities and Innovation in Sport(L-VIS EA 7428)University of Lyon 1Villeurbanne 69622France Institute of Sport Faculty of EducationHealth and Well-beingUniversity of WolverhamptonWs13BDUK Faculty of Sport Sciences Universitéde Reims Champagne-ArdenneReims 51096France Research Team of Psychology of Ages of Life(EA 2114) University Francois RabelaisTours 37041France CETAPS(EA 3832) Normandie UniversitéMont Saint Aignan 76821France

Purpose:This study investigated emotion-performance relationships in rugby *** identified which emotions rugby players experienced and the extent to which these emotions were associated with performance,considering how emotions unfold over the course of a game,and whether the game was played at home or ***:Data were gathered from 22 professional male rugby union players using auto-confrontation interviews to help identify situations within games when players experienced intense *** assessed the intensity of emotions experienced before each discrete performance and therefore could assess the emotion-performance relationship within a ***:Players identified experiencing intense emotions at 189 *** in rugby union rated the quality of each performance at these 189 time-points on a visual analog scale.A Linear Mixed Effects model to investigate emotion-performance relationships found additive effects of game location,game time,and emotions on individual ***:Results showed 7 different pre-performance emotions,with high anxiety and anger associating with poor *** research should continue to investigate emotion-performance relationships during performance using video-assisted recall and use a measure of performance that has face validity for players and coaches alike.

关键词： Emotion Game location Game time Linear Mixed Effects model Multilevel analysis Performance assessment Team sport

Improving cell transplantation by understanding and manipulating the phagocytic activity of peripheral glia

在线全文

学校读者我要写书评

暂无评论

Neural Regeneration Research 2022年第2期17卷 313-314页

作者： Lynn Nazareth James st John Jenny Ekberg Menzies Health Institute Queensland Griffith UniversitySouthportAustralia Clem Jones Centre for Neurobiology and stem Cell Research Griffith UniversityNathanAustralia Griffith Institute for Drug Discovery Griffith UniversityNathanAustralia

One of the key challenges in neuroscience is that the central nervous system(CNS;the brain and spinal cord), is largely unable to regene rate after injury. One factor contributing to this lack of repair is the accumulation of cellular and myelin debris at the site of injury. The debris is not efficiently phagocytosed and can persist for years after the initial injury, resulting in an inflammatory environment which inhibits axonal regrowth(Lutz and Barres, 2014).

关键词： debris contributing injury

Enlarged and uncommon indications for ALPPS-where do we stand?

在线全文

学校读者我要写书评

暂无评论

Hepatobiliary Surgery and Nutrition 2022年第5期11卷 743-745页

作者： stéphanie Truant Department of Digestive Surgery and Transplantation CHU LilleUniv.LilleLilleFrance CANTHER Laboratory“Cancer Heterogeneity Plasticity and Resistance to Therapies”UMR-S1277Team“MucinsCancer and Drug Resistance”LilleFrance

Associating liver partition and portal vein ligation for staged hepatectomy(ALPPS)has been introduced in 2012 as an innovative surgical option that offers patients curative resection in otherwise nonresectable *** the high morbi-mortality rates and poor oncologic outcome in the early experience,indications of ALPPS have been restricted to young patients with colorectal liver metastases(CRLM)following the first report on the international ALPPS registry(1).ALPPS further gained in safety after the first International ALPPS Meeting held in 2015(2)in Hamburg,which prompted surgeons to better patient selection by chemotherapy(3),technical refinements toward less invasive ALPPS procedures(4),and better inter-stage decision making(5).

关键词： ALPPS indications mortality

Bubbles and incentives:an experiment on asset markets

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Economic and Political studies 2021年第1期9卷 68-89页

作者： stéphane Robin Katerina stráznická Marie Claire Villeval Grenoble INP Institute of Engineering and Management Grenoble Applied Economics Laboratory(GAEL)University of Grenoble AlpesGrenobleFrance French National Centre for Scientific Research(CNRS) ParisFrance National Institute for Agricultural Research(INRA) ParisFrance Clermont Research Management(CleRMA) University of Clermont AuvergneClermont-FerrandFrance University of Lyon LyonFrance Group of Economic Analysis and Theory(GATE) French National Centre for Scientific Research(CNRS)EcullyFrance Institute of Labour Economics(IZA) BonnGermany

We explore the effects of competitive incentives and of their time horizon on the evolution of both asset prices and trading activity in experimental asset *** compare(i)a no-bonus treatment;(ii)a short-term bonus treatment in which bonuses are assigned to the best performers at the end of each trading period;(iii)a long-term bonus treatment in which bonuses are assigned to the best performers at the end of the 15 periods of the *** find that the existence of bonus contracts does not increase the likelihood of bubbles but it affects their severity,depending on the time horizon of *** with longterm bonus contracts experience lower price deviations and a lower turnover of assets than markets with either no bonuses or long-term bonus ***-term bonus contracts increase price deviations but only when markets include a higher share of male *** the individual level,the introduction of bonus contracts increases the trading activity of males,probably due to their higher competitiveness.

关键词： Asset market bubbles incentives bonuses risk attitudes experiment