1,428 research outputs found
Imagerie encéphalique dans la maladie de Fabry
No full textLa maladie de Fabry est une maladie de surcharge lysosomale de transmission génétique liée à l X à l origine de lésions encéphaliques observables en imagerie par résonnance magnétique. Les associations que ces atteintes présentent entre elles et avec certains paramètres clinico-biologiques restent à explorer. Les données de 86 patients du centre de référence national de la maladie de Fabry furent ainsi analysées. La plupart des associations connues dans la population générale sont retrouvées chez nos patients témoignant de voies physiopathologiques communes. L hypersignal T1 des pulvinars est bien une exclusivité masculine présente chez un quart des hommes, et qui est fréquemment mais pas systématiquement associé à une cardiomyopathie (64%) ou à une insuffisance rénale sévère (71%). Les accidents vasculaires cérébraux sont essentiellement de type lacunaire avec une localisation vertébro-basilaire plus fréquente que dans la population générale (35% versus 20%) mais non majoritaire. La longueur du tronc basilaire n est pas affectée par la maladie de Fabry, tandis que son diamètre moyen est plus affecté que son diamètre maximal. La leucoencéphalopathie et les lacunes sont plus associées à une augmentation du diamètre de l artère cérébrale moyenne qu à celui du tronc basilaire. La susceptibilité de chaque sexe au déficit enzymatique notamment au niveau du système cérébro-vasculaire et du cœur reste à déterminer par des analyses statistiques multivariées.Fabry disease is a lysosomal storage X-linked disorder leads to brain abnormalities in magnetic resonance imaging. The associations that these lesions present between them and with certain clinical or biological parameters remain to be explored. The data of 86 patients of the national reference center of Fabry disease were thus analyzed. Most of the associations known in the general population are also detected among our patients indicating common physiopathological pathways. T1 hyperintensity of pulvinars is indeed a masculine exclusivity present among a quarter of men, and which is frequently but not systematically associated with cardiomyopathy (64%) or with a endstage renal failure (71%). Most strokes are lacunar type with a vertebrobasilar location more frequent than in the general population (35% versus 20%). The length of the basilar trunk is not affected by Fabry disease, while its average diameter is more affected than its maximum diameter. Leukoencephalopathy and lacunar strokes are more associated with an increase of the middle cerebral artery diameter than with the one of the basilar artery. Sensitivity of every sex to the enzymatic deficit notably at the level of the cerebrovascular system and of the heart remains to be determined by multivariate statistical analysis.ST QUENTIN EN YVELINES-BU (782972101) / SudocSudocFranceF
The author as actor: a defense of Quentin Skinner
No full textIn this thesis, I defend Quentin Skinner's work against some criticisms raised by three of his interlocutors: John Keane, Kennet Minogue, and Joseph Femia. All three of these critics take issue with Skinner's author-centered approach to the historical interpretation of texts. Femia, invoking Roland Barthes 'death of the author' thesis, argues that Skinner's attempt to recover the intentions of authors is impossible. While Minogue and Keane do not dispute the possibility of recovering an author's intentions, they question the unity of such an enterprise. In order to answer Femia's criticism of Skinner, I draw an analogy between Skinner's figure of the author, and Arendt's figure of the political actor. I argue that just as it is possible for someone to know what a political actor is doing in performing a political act, it is similarly possible for an intellectual historian to understand what political acts an author was doing in writing his or her text. To refute Minogue's and Keane's claims that a Skinnerian approach to intellectual history is of no use to the political theorist, I point to three examples of how Skinner's recovery of forgotten political discourses have been applied to contemporary debates in political theory.Graduat
Drawing Sacred Forests and Courtyards in South Benin
No full texthttps://drawingmatter.org/drawing-sacred-forests-and-courtyards-in-south-benin/The following conversation between the editors of Accattone and Quentin Nicolaï was first published in Accattone 6 (2019). It documents research carried out by Quentin Nicolaï in Abomey, Benin, between January 2014 and June 2018.Drawing Matter would like to thank the author and the magazine’s editors for allowing us reproduce the essay on www.drawingmatter.org.info:eu-repo/semantics/nonPublishe
Features of auteur cinema in Quentin Tarantino's works
No full textMultimediju komunikācijaInformācijas un komunikācijas zinātnesMultimedia CommunicationInformation and Communication SciencesBakalaura darba tēmas nosaukums ir “Autorkino iezīmes Kventina Tarantīno daiļradē.”
Pētījumā izvirzītā hipotēze apgalvo, ka režisora Kventina Tarantīno (Quentin Tarantino) filmās ir
atrodamas autorkino iezīmes.
Bakalaura darba mērķis ir noskaidrot autorkino iezīmes Kventina Tarantīno sešās
ietekmīgākajās un skatītāju atzītākajās filmās, kuras tika atlasītas pēc Internacionālās filmu datu
bāzes. Darbā tiek apskatītas un analizētas sešas no Kventina Tarantīno filmām, kuras ir “Trakie
suņi” (Reservoir Dogs), “Lubene” (Pulp Fiction), “Nogalināt Bilu: 1. daļa” (Kill Bill: Vol. 1) un
“Nogalināt Bilu: 2. daļa” (Kill Bill: Vol. 2), kas pēc paša režisora uzskatiem ir, viena vesela, filma
sadalīta divās daļās, kā arī filmas “Bēdīgi slavenie mērgļi” (Inglorious Bastards), “Atsvabinātais
Džango” (Django Unchained) un “Reiz Holivdā” (Once Upon a Time... in Hollywood).
Darba mērķa izpildei teorētiskajā daļā tika aplūkots autorkino jēdziens, Holivudas
klasiskais kino stils, kino valoda, kā arī Kventina Tarantīno biogrāfija, lai palīdzētu izprast un
analizēt atlasītās filmas. Par pētījuma metodi tika izvēlēta kvalitatīvā kontentanalīze.The title of the bachelor's thesis is "Features of auteur cinema in Quentin Tarantino's
works" The study’s hypothesis states that directors Quentin's Tarantino's films have the features
of an author.
The purpose of the bachelor's thesis is to find out the influence of author cinema on Quentin
Tarantino's six most influential and audience-recognized films selected from the International Film
Database. Work is discussed and analyzed in six of Quentin Tarantino films, which are “Reservoir
Dogs”, “Pulp Fiction”, “Kill Bill: Vol. 1” and “Kill Bill: Vol. 2”, which the director says is a whole
movie divided into two parts, as well as “Inglorious Bastards”, “Django Unchained” and “Once
Upon a Time ... in Hollywood”.
The theoretical part of the paper explored the concept of author cinema, Hollywood classic
cinema, film language, and the biography of Quentin Tarantino to help understand and analyze
selected films. Qualitative content analysis was chosen as the research method
Saint-Quentin et sa région
No full textThe author insists on the seriousness of the trade depression in Saint-Quentin last years (ten per cent workmen out of work, unemployed period above eleven months.). Metallurgy is the principal industry and «Motobécane» the first firm, making the famous «Mobylette» since 1951. Therefore, the difficulties of «Motobécane», of the engineering industry, have effects on the social situation. The town is, from now on, the starting point of an urban emigration. Old fortress on the right bank, protecting the crossing of the river Somme, Saint-Quentin has been destroyed during the first World War and rebuilt without large transformation. The town keeps an heptagonal shape with cross-roads on the both sides of the valley, canal and railway. The attraction area is, to-day, smaller than the Vermandois, very industrialized and populated region. For the future, Saint-Quentin shall have to expect : the «A-26 » motorway, the négociation of an employment agreement («Contrat de Bassin d'Emploi») and, later, the construction of the large-gauge canal «Seine-Nord» from Compiègne to Valenciennes.L'auteur insiste sur la gravité de la crise de remploi à Saint-Quentin aujourd 'hui (taux de chômage supérieur à 1 0%, durée moyenne de chômage supérieure à 11 mois). L'industrie est dominée par la métallurgie et celle-ci par la firme «MOTOBECANE» qui fabrique depuis 1951 les fameuses «mobylettes». Aussi les difficultés de Motobécane ou de l'industrie de la manutention rejaillissent-elles immédiatement sur la situation sociale. La ville est désormais le point de départ d'un véritable exode urbain. Ancienne place forte gardant, sur la rive droite , le passage de la Somme, presque complètement détruit en 1918, mais reconstruit largement à l'identique, St-Quentin a hérité d'une double structure heptagonale et en «patte d'oie» de part et d'autre de la coupure majeure de la vallée de la Somme (canal et voie ferrée). Son aire d'attraction s'est réduite par rapport à la région qui l'entoure, le Vermandois, relativement peuplé et industrialisé lui-même. Pour l'avenir, St-Quentin doit compter surtout, sur l'arrivée de l'autoroute A 26 et sur les efforts qui s'inscriront dans un «Contrat de Bassin d'Emploi», et, à long terme sur la liaison fluviale à grand gabarit «Seine-Nord» utilisant le tracé du canal de St-Quentin.Oudart Paul. Saint-Quentin et sa région. In: Hommes et Terres du Nord, 1981/2. Spécial Picardie. pp. 43-56
A Hundred Years of Photo Wallets:Quentin Blake Centre for Illustration's Book of the Month
No full textAn interview with Annebella Pollen, author of More Than a Snapshot: A Visual History of Photo Wallets, which was selected as the May 2023 Book of the Month by the Quentin Blake Centre for Illustration
Quentin Meillassoux: Facticity and Scientific Methods
No full textThis paper examines Quentin Meillassoux’s critique of correlationism, specifically focusing on the Principle of Factuality and the mathematization of hyperchaos as tools to access the "ancestral." While acknowledging Meillassoux’s significance in overcoming Kantian limits, the author argues that his refusal to return to metaphysics stems from political hesitation rather than ontological necessity. The text critiques the concept of the "God to come" as an ideological compromise. Instead, the author advocates for an "experimental metaphysics" grounded in scientific method, positing that science offers rational access to the absolute and effectively resolving the tension between facticity and the thing-in-itself
Quentin Meillassoux: Facticity and Scientific Methods
No full textThis paper examines Quentin Meillassoux’s critique of correlationism, specifically focusing on the Principle of Factuality and the mathematization of hyperchaos as tools to access the "ancestral." While acknowledging Meillassoux’s significance in overcoming Kantian limits, the author argues that his refusal to return to metaphysics stems from political hesitation rather than ontological necessity. The text critiques the concept of the "God to come" as an ideological compromise. Instead, the author advocates for an "experimental metaphysics" grounded in scientific method, positing that science offers rational access to the absolute and effectively resolving the tension between facticity and the thing-in-itself
A Hundred Years of Photo Wallets:Quentin Blake Centre for Illustration's Book of the Month
No full textAn interview with Annebella Pollen, author of More Than a Snapshot: A Visual History of Photo Wallets, which was selected as the May 2023 Book of the Month by the Quentin Blake Centre for Illustration
Global reach of over 20 years of experience in the patient-centered Fabry Registry: Advancement of Fabry disease expertise and dissemination of real-world evidence to the Fabry community
No full textInternational audienceFabry disease (FD, α-galactosidase A deficiency) is a rare, progressive, complex lysosomal storage disorder affecting multiple organ systems with a diverse spectrum of clinical phenotypes, particularly among female patients. Knowledge of its clinical course was still limited in 2001 when FD-specific therapies first became available and the Fabry Registry (NCT00196742; sponsor: Sanofi) was initiated as a global observational study. The Fabry Registry has now been operational for over 20 years, overseen by expert Boards of Advisors, and has collected real-world demographic and longitudinal clinical data from more than 8000 individuals with FD. Leveraging the accumulating evidence base, multidisciplinary collaborations have resulted in the creation of 32 peer-reviewed scientific publications, which have contributed to the greatly expanded knowledge on the onset and progression of FD, its clinical management, the role of sex and genetics, the outcomes of enzyme replacement therapy with agalsidase beta, and prognostic factors. We review how the Fabry Registry has evolved from its inception to become the largest global source of real-world FD patient data, and how the generated scientific evidence has helped to better inform the medical community, individuals living with FD, patient organizations, and other stakeholders. The patient-centered Fabry Registry fosters collaborative research partnerships with the overarching goal of optimizing the clinical management of patients with FD and is well positioned to add to its past achievements
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