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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-Robin 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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Optical coherence tomography angiography at the acute phase of optic disc edema

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Eye and Vision 2018年第1期5卷 144-150页

作者： Marie-Bénédicte Rougier Mélanie Le Goff jean-françois Korobelnik Service d’Ophtalmologie CHU de BordeauxBordeauxFrance Bordeaux Population health Research Center Team LEHAUniversity BordeauxINSERMUMR 1219F-33000 BordeauxFrance

Background:The differential diagnosis of optic disc edema at the acute phase can be *** angiography(OCTA)is a new technology allowing the visualization of the peripapillary vascular network and optic disc *** peripapillary network alterations of glaucoma and chronic non-arteritic anterior ischemic optic neuropathy(NAION)were ***,no OCTA studies on acute optic disc edema from various *** aim of this project was to use OCTA to demonstrate the vascular changes the optic nerve head of various types of optic disc edema at the acute ***:In this retrospective study,patients with non-arteritic anterior ischemic optic neuropathy(NAION),papillitis or papilledema were *** patient was imaged using the AngioPlex^(TM)CIRRUS^(TM)hD-OCT device(model 5000,Carl Zeiss Meditec,Inc.,Dublin,USA)with a scanning area of 6×6 mm^(2) centered on the optic disc.A morphological analysis of the peripapillary network was *** some patients with unilateral optic disc edema,a quantitative analysis was performed using a swept-source OCT-A system(PLEX®Elite 9000,Carl Zeiss Meditec,Inc.,Dublin,USA).Vessel perfusion density and flux index of the peripapillary area were ***:Eight eyes with NAION(4 patients),12 eyes with papillitis(6 patients)and 25 eyes with papilledema(13 patients)were *** apparent disappearance or moderate pattern alteration of the peripapillary capillary vessels were observed in patients with NAION or papillitis,*** papilledema,the capillaries at the surface of the optic disc were dilated and tortuous,but no peripapillary network pattern changes were *** quantitative analysis did not show any difference of peripapillary network between NAION and healthy *** papillitis,the flux index was higher in inflammatory eyes compared to the healthy eyes in average(p=0.03).Conclusion:At the acute phase,the morphological analysis of OCT-A appeared to be more useful than the q

关键词： Optical coherence tomography angiography Optic nerve head Edema NAION Microvascular network And morphology

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From Control Theory to Gravitational Waves

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Advances in Pure Mathematics 2024年第2期14卷 49-100页

作者： jean-francois Pommaret CERMICS Ecole des Ponts Paris Tech Paris France

When D:ξ→η is a linear ordinary differential (OD) or partial differential (PD) operator, a “direct problem” is to find the generating compatibility conditions (CC) in the form of an operator D1:η→ξ such that Dξ = η implies D1η = 0. When D is involutive, the procedure provides successive first-order involutive operators D1,...,Dn when the ground manifold has dimension n. Conversely, when D1 is given, a much more difficult “inverse problem” is to look for an operator D:ξ→η having the generating CC D1η = 0. If this is possible, that is when the differential module defined by D1 is “torsion-free”, that is when there does not exist any observable quantity which is a sum of derivatives of η that could be a solution of an autonomous OD or PD equation for itself, one shall say that the operator D1 is parametrized by D. The parametrization is said to be “minimum” if the differential module defined by D does not contain a free differential submodule. The systematic use of the adjoint of a differential operator provides a constructive test with five steps using double differential duality. We prove and illustrate through many explicit examples the fact that a control system is controllable if and only if it can be parametrized. Accordingly, the controllability of any OD or PD control system is a “built in” property not depending on the choice of the input and output variables among the system variables. In the OD case and when D1 is formally surjective, controllability just amounts to the formal injectivity of ad(D1), even in the variable coefficients case, a result still not acknowledged by the control community. Among other applications, the parametrization of the Cauchy stress operator in arbitrary dimension n has attracted many famous scientists (G. B. Airy in 1863 for n = 2, J. C. Maxwell in 1870, E. Beltrami in 1892 for n = 3, and A. Einstein in 1915 for n = 4).

关键词： Differential Operator Differential Sequence Killing Operator Riemann Operator Bianchi Operator Cauchy Operator Control Theory Controllability Elasticity General Relativity

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Astral Actions on Allais’ Pendulum Apparently Inexplicable by Classical Factors: A Point of the Situation

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Journal of Modern Physics 2024年第9期15卷 1375-1408页

作者： jean-Bernard Deloly Independant Researcher Epinay sur Orge France

1) The observation by Allais of the precession of pendulums from 1954 to 1960 highlighted regularities of astral origin an in-depth analysis of which showed that, apparently, no classical phenomenon can explain them. These regularities were diurnal waves whose periods are characteristic of astral influence (the main ones being 24 h and 24 h 50 min), annual and semi-annual components, and a multi-annual component of approximately 6 years, an influence of Jupiter being a very good candidate to explain it. 2) Allais had experimentally established that all these astral influences were expressed globally on the pendulum by an action tending to call back its plane of oscillation towards a direction variable in time, and which ovalized its trajectory. In 2019 the observation of 2 pendulums in horodnic (Romania), thanks to the use of an automatic alidade, made it possible to identify the main mechanism that, very probably, acted on the pendulum to achieve this result. This perturbation model, called “linear anisotropy”, is characterized by its “coefficient of anisotropy” η, and by the azimuth of its “direction of anisotropy”. The composition of 2 linear anisotropies is always a linear anisotropy. 3) In the search for the phenomena which could be at the origin of all what precedes, the fact that they must create an ovalization immediately eliminates some of them. 4) We have calculated the values of η corresponding to the 24 h and 24 h 50 min waves both for the observations in horodnic and the Allais observations. The order of magnitude (some 10−7) is effectively the same in both cases. 5) Mathematically, the regularities discovered may result of a new force field but also, as Allais proposes, from the creation, under the astral influences, of a local anisotropy of the medium in which the pendulum oscillates. In the first case the length of the pendulum is involved, in the second one not. The data available do not make it possible to decide. 6) The joint exploitation, in mech

关键词： Allais Effect Pendulum Lunisolar Influence Jupiter Influence Lunar and Solar Eclipses Syzygies Sunspots Solar Cycles

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The Njiki’s Fundamental Theorem-Definition on Fractions in the Mathematical Set ℚ and by Extension in ℝ and ℂ, for the Purpose of Leading to the Construction of Some Algebraic Structures as Its Theoretical Applications and for the Practical Ones

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American Journal of Computational Mathematics 2024年第3期14卷 358-379页

作者： jean Claude Njiki Independent Researcher Yaound Cameroon

The purpose of the research in the NJIKI’s fundamental ThEOREM-DEFINITION on fractions in the mathematical set ℚand by extension in ℝand ℂand in order to construct some algebraic structures is about the proved EXISTENCE and the DEFINITION by NJIKI of two INNOVATIVE, IMPORTANT and TEAChABLE operations of addition or additive operations, in ℚ, marked ⊕and +α,β, and taken as VECTORIAL, TRIANGULAR, of ThREE or PROPORTIONAL operations and in order to make ThEM not be different from the RATIONAL ONE, +, but to bring much more and new information on fractions, and, by extension in ℝand ℂ. And the very NJIKI’s fundamental ThEOREM-DEFINITION having many APPLICATIONS in the everyday life of the hUMAN BEINGS and without talking about computer sciences, henceforth being supplied with very interesting new ALGORIThMS. And as for the work done in the research, it will be waiting for its extension to be done after publication and along with the research results concerned.

关键词： Operations of Addition Additive Operations in ℚ Laws in ℚ ThEOREM-DEFINITION External Multiplication

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Review of the development and uses of hard grade asphalts in france

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Journal of Road Engineering 2021年第1期1卷 73-79页

作者： jean-francois Corte Directorate of Transport Infrastructures Ministry of TransportLa Defense 92055France

hard grade paving asphalts,i.e.,having a penetration at 25C lower than 25 mm/10,have experienced,in france,a significant development over the last forty years,linked to the development of high modulus asphalt concrete for rehabilitation then for new construction of road *** the first part,this paper provides a review of the evolution of asphalts in france over the past sixty years towards the use of harder penetration grades and a limitation of the evolution of the asphalt in the mixing plant to avoid early cracking of the *** recommendations in relation to climatic conditions are *** characteristics of different hard asphalts produced in france are presented including data on the effect of ageing tests rolling thin film oven test(RTFOT)and pressure ageing vessel(PAV).hardening of hard asphalt after RTFOTþPAV is comparable to that of softer *** performance related to low temperature cracking is less which limits the use of plain hard asphalt in pavements subjected to severe winter conditions and requires polymer *** specifications for hard grade paving asphalts based on traditional empirical tests included in the European standard developed in the mid 2000s and the choices made in france are presented.A comparison of the specifications for hard grade paving asphalts in the European standards and of those proposed for China shows that the European specifications are more stringent as regards consistency and evolution after ageing tests.

关键词： Asphalt hard grade Rheology Standard

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Structural Setting of the South-West Cameroon Using Satellite Potential Field Derived from SGG-UGM-2 Gravity Data

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Journal of Geoscience and Environment Protection 2024年第8期12卷 43-61页

作者： jean Aimé Mono Basical Science Department Advanced Technical Teacher Training School University of Douala Douala Cameroon

This study aims to improve knowledge of the structure of southwest Cameroon based on the analysis and interpretation of gravity data derived from the SGG-UGM-2 model. A residual anomaly map was first calculated from the Bouguer anomaly map, which is strongly affected by a regional gradient. The residual anomaly map generated provides information on the variation in subsurface density, but does not provide sufficient information, hence the interest in using filtering with the aim of highlighting the structures affecting the area of south-west Cameroon. Three interpretation methods were used: vertical gradient, horizontal gradient coupled with upward continuation and Euler deconvolution. The application of these treatments enabled us to map a large number of gravimetric lineaments materializing density discontinuities. These lineaments are organized along main preferential directions: NW-SE, NNE-SSW, ENE-WSW and secondary directions: NNW-SSE, NE-SW, NS and E-W. Euler solutions indicate depths of up to 7337 m. Thanks to the results of this research, significant information has been acquired, contributing to a deeper understanding of the structural composition of the study area. The resulting structural map vividly illustrates the major tectonic events that shaped the geological framework of the study area. It also serves as a guide for prospecting subsurface resources (water and hydrocarbons). .

关键词： SGG-UGM-2 Model horizontal Gradient Bouguer Anomalies Potential Field Data

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Risk factors for running-related injuries:An umbrella systematic review

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Journal of Sport and health Science 2024年第6期13卷 793-804页

作者： Clara Knierim Correia jean Marlon Machado Fabio hech Dominski Marcelo Peduzzi de Castro heiliane de Brito Fontana Caroline Ruschel College of health and Sport Science State University of the Santa CatarinaFlorianopolis 88080-350Brazil Fisiolab Institut Florianopolis 88015-310Brazil Biological Sciences Centre Federal University of Santa CatarinaFlorianopolis 88040-900Brazil

Purpose:This umbrella systematic review(SR) of SRs and meta-analysis seeks to comprehensively synthesize existing literature to identify and consolidate the diverse range of risk factors contributing to running-related injuries(RRIs).Methods:Systematic searches were conducted on June 28,2023,across Web of Science,SPORTDiscus,Scopus,PubMed,and Cochrane *** included SRs,whether accompanied by meta-analyses or not,that focused on investigating risk factors for RRIs within observational *** methodological quality of the SRs was evaluated using the Assessing the Methodological Quality of Systematic Reviews Ⅱ.To assess the extent of overlap across reviews,the corrected covered area metric was ***:From 1509 records retrieved,13 SRs were *** degree of overlap between SRs was low(4%),and quality varied from critically low(n=8) to low(n=5).Two hundred seven outcomes assessed in 148 primary studies were identified as being associated with the occurrence of *** effect sizes of the associations for which risk measures were reported(n=131) were classified as large(n=30,23%),medium(n=38,29%),small(n=48,37%) or no effect(n=15,11%).Running/training characteristics,health and lifestyle factors,along with morphological and biomechanical aspects,exhibit large effect sizes in increasing the risk for ***:Drawing from the outcomes of the low-quality SRs and associations with large effect sizes,our findings indicate that running/training characteristics and health and lifestyle factors,as well as morphological and biomechanical aspects,are all implicated in elevating the risk of RRIs,emphasizing the multifactorial basis of injury incidence in *** the low quality and heterogeneity of SR,individual findings warrant cautious interpretation.

关键词： Umbrella review Runners Injury Risk factor

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Life Strategy Traits of the Liana Sericostachys scandens Spreading in the Montane Forests in the Kahuzi-Biega National Park (DR Congo)

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Journal of Mountain Science 2012年第5期9卷 665-675页

作者： Masumbuko Ndabaga Céphas herpigny Basile Barbier Nicolas habiyaremye Muhashy franois Lejoly jean Meerts Pierre Université Officielle de Bukavu PoBox 570 Bukavu DR Congo Laboratoire d'Ecologie Végétale et Biogéochimie CP 244 Université Libre de Bruxelles Av FD Roosevelt 50 B-1050 Bruxelles Belgium Botany and Computational Plant Architecture IRD - Marie Curie. 34398 Montpellier cedex 5 France Institut Royal des Sciences Naturelles de Belgique Rue Vautier 29 1000 Bruxelles Belgium herbarium de l'Université Libre de Bruxelles 50 Avenue F. Roosevelt - CP 169 B-1050 Bruxelles Belgium

Sericostaehys seandens is a monocarpic and heliophilous liana, native in tropical African forests. In the montane forests of the Kahuzi-Biega National Park （KBNP）（East of DR Congo）, it has been expanding very strongly for a decade, and is currently considered as having negative impacts on biodiversity conservation. In this paper, we test if S. scandens differs from three co-occurring, native, non spreading lianas （Gouania longispicata, Tacazzea apiculata and Adenia bequaertii） for functional traits which might influence plant expansion. For leaf traits （SLA, dry matter content, nitrogen concentration）, S. scandens did not show extreme values compared to those of the three other lianas. In contrast, S. scandens had much higher biomass allocation to sexual reproduction. It also differs from the three other lianas for its reproductive strategy that combines both vegetative propagation and sexual reproduct/on, and propagule dispersal by wind. Moreover, S. scandens has larger leaves and a greater number of lateral branches per unit stem length. It is argued that the particular combination of functional traits exhibited by *** may in part explain its propensity to behave as an opportunistic weed in the disturbed areas in the montane forests of Kahuzi-Biega.

关键词： Life strategy Sericostachys seandens montane forest Kahuzi-Biega National Park functional traits Native expanding species.

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Severe asthma exacerbation:Changes in patient characteristics,management,and outcomes from 1997 to 2016 in 40 ICUs in the greater Paris area

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Journal of Intensive Medicine 2024年第2期4卷 209-215页

作者： Romy Younan jean Loup Augy Bertrand hermann Bertrand Guidet Philippe Aegerter Emmanuel Guerot Ana Novara Caroline hauw-Berlemont Amer hamdan Clotilde Bailleul francesca Santi jean-Luc Diehl Nicolas Peron Nadia Aissaoui Intensive Care Unit AP-HPGeorges Pompidou European HospitalUniversitéde ParisParisFrance Intensive Care Unit AP-HPSaint Antoine HospitalUniversités de SorbonneUniversitéPierre et Marie CurieParisFrance INSERM U1136 ParisFrance Versailles Saint-Quentin-en-Yvelines University INSERM U1018Groupe Interrégional de Recherche Clinique et d'InnovationÎle-de-FranceFrance Respiratory Medicine Department AP-HPGeorges Pompidou European HospitalUniversitéde ParisParisFrance Innovative Therapies in hemostasis INSERM UMR-S1140Universitéde ParisParisFrance Intensive Care Unit and Biosurgical Research Lab(Carpentier Foundation) AP-HPGeorges Pompidou European HospitalParisFrance Paris-Cardiovascular-Research-Center INSERM U970ParisFrance

Background Despite advances in asthma treatments,severe asthma exacerbation(SAE)remains a life-threatening condition in adults,and there is a lack of data derived from adult patients admitted to intensive care units(ICUs)for *** current study investigated changes in adult patient characteristics,management,and outcomes of SAE over a 20-year period in 40 ICUs in the greater Paris *** In this retrospective observational study,admissions to 40 ICUs in the greater Paris area for SAE from January 1,1997,to December 31,2016 were *** primary outcome was the proportion of ICU admissions for SAE during 5-year *** outcomes were ICU and hospital mortality,and the use of mechanical ventilation and *** analysis was performed to assess factors associated with ICU *** A total of 7049 admissions for SAE were *** each 5-year period,the proportion decreased over time,with SAE accounting for 2.84%of total ICU admissions(n=2841)between 1997 and 2001,1.76%(n=1717)between 2002 and 2006,1.05%(n=965)between 2007 and 2011,and 1.05%(n=1526)between 2012 and *** median age was 46 years(interquartile range[IQR]:32–59 years),55.41%were female,the median Simplified Acute Physiology Score II was 20(IQR:13–28),and 19.76%had mechanical *** use of mechanical ventilation remained infrequent throughout the 20-year period,whereas the use of catecholamine *** and hospital mortality rates *** associated with ICU mortality were renal replacement therapy,catecholamine,cardiac arrest,pneumothorax,acute respiratory distress syndrome,sepsis,and invasive mechanical ventilation(IMV).Non-survivors were older,had more severe symptoms,and were more likely to have received *** ICU admission for SAE remains uncommon,and the proportion of cases decreased over *** a slight increase in symptom severity during a 20-year period,ICU and hospital mortality *** requirin

关键词： Epidemiology Intensive care unit Mechanical ventilation Severe asthma exacerbation

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