53 research outputs found
Exploration de mon processus de création : études sur les effets de vérité au sein des productions médiatiques
Ce mĂ©moire prĂ©sente le travail de rĂ©flexion qui a conduit Ă la crĂ©ation dâune Ćuvre nommĂ©e FrontiĂšres quotidiennes. La recherche prend pour thĂšme initial le quotidien dâun artiste. Par une sĂ©rie de questions, lâauteur sâinterroge sur la notion de vĂ©ritĂ© en art. Il passe en revue les Ă©tapes de crĂ©ation de son projet, de lâidĂ©e Ă lâexposition. Il positionne son travail en relation avec dâautres Ćuvres rĂ©alisĂ©es par des artistes ayant une grande renommĂ©e. En conclusion, ce mĂ©moire rĂ©vĂšle la vĂ©ritĂ©. Elle tient au fait quâil existe de multiples vĂ©ritĂ©s. Les artifices mis en place par lâartiste dans son projet amĂšnent le spectateur Ă construire un moteur dâinfĂ©rence qui oriente sa perception et son interprĂ©tation du rĂ©el. Lâauteur incite le spectateur Ă porter un regard critique sur les images numĂ©riques qui lui sont soumises par les mĂ©dias actuels
Cardiac outcomes in adults with supravalvar aortic stenosis
Aims Supravalvar aortic stenosis is a rare form of left ventricular outflow tract obstruction that is often progressive in childhood. Little data are available on outcomes in the adult population. Our aim was to define cardiac outcomes in adults with supravalvar aortic stenosis. Methods and results This is a multicentre retrospective study of cardiac outcomes in adults (â„18 years) with supravalvar aortic stenosis. We examined: (i) adverse cardiac events (cardiovascular death, myocardial infarction, stroke, heart failure, sustained arrhythmias, and infective endocarditis) and (ii) the need for cardiac surgery in adulthood. One hundred and thirteen adults (median age at first visit 19 years; 55% with Williams-Beuren syndrome; 67% with surgical repair in childhood) were identified. Adults without Williams-Beuren syndrome had more severe supravalvar aortic stenosis and more often associated left ventricular outflow tract obstructions (P < 0.001). In contrast, mitral valve regurgitation was more common in patients with Williams-Beuren syndrome. Eighty-five per cent of adults (96/113) had serial follow-up information (median follow-up 6.0 years). Of these patients, 13% (12/96) had an adverse cardiac event and 13% (12/96) had cardiac operations (7 valve repair or replacements, 4 supravalvar aortic stenosis repairs, 1 other). Cardiac surgery was more common in adults without Williams-Beuren syndrome (P = 0.007). Progression of supravalvar aortic stenosis during adulthood was rare. Conclusion Adults with supravalvar aortic stenosis remain at risk for cardiac complications and reoperations, while progression of supravalvar aortic stenosis in adulthood is rare. Valve surgery is the most common indication for cardiac surgery in adulthoo
Loss-of-function mutations in the X-linked biglycan gene cause a severe syndromic form of thoracic aortic aneurysms and dissections.
Thoracic aortic aneurysm and dissection (TAAD) is typically inherited in an autosomal dominant manner, but rare X-linked families have been described. So far, the only known X-linked gene is FLNA, which is associated with the periventricular nodular heterotopia type of Ehlers-Danlos syndrome. However, mutations in this gene explain only a small number of X-linked TAAD families.
We performed targeted resequencing of 368 candidate genes in a cohort of 11 molecularly unexplained Marfan probands. Subsequently, Sanger sequencing of BGN in 360 male and 155 female molecularly unexplained TAAD probands was performed.
We found five individuals with loss-of-function mutations in BGN encoding the small leucine-rich proteoglycan biglycan. The clinical phenotype is characterized by early-onset aortic aneurysm and dissection. Other recurrent findings include hypertelorism, pectus deformity, joint hypermobility, contractures, and mild skeletal dysplasia. Fluorescent staining revealed an increase in TGF-ÎČ signaling, evidenced by an increase in nuclear pSMAD2 in the aortic wall. Our results are in line with those of prior reports demonstrating that Bgn-deficient male BALB/cA mice die from aortic rupture.
In conclusion, BGN gene defects in humans cause an X-linked syndromic form of severe TAAD that is associated with preservation of elastic fibers and increased TGF-ÎČ signaling.Genet Med 19 4, 386-395
LâaprĂšs-mobilisation : le Mouvement du 20 FĂ©vrier au Maroc, de la dĂ©sillusion au redĂ©ploiement de lâengagement
Cet article analyse lâimpact du Mouvement du 20 FĂ©vrier (M20F) au Maroc qui Ă©merge dans le sillage des soulĂšvements arabes de 2011 en se focalisant sur le devenir des participants dans lâaprĂšs-mobilisation. Lâessoufflement de la dynamique protestataire et la mise en veille du mouvement ont pu sâaccompagner dâun sentiment de dĂ©sillusion chez les militants. Ce sentiment nâa pas que favorisĂ© la dĂ©mobilisation, mais aussi le redĂ©ploiement de lâengagement Ă dâautres Ă©chelles, suivant dâautres temporalitĂ©s, vers les « structures de rĂ©manence » (abeyance structures) de lâespace protestataire. LâĂ©preuve du M20F a indĂ©niablement marquĂ© une gĂ©nĂ©ration de militants qui sâinscrivent dans la continuitĂ© des combats de leurs aĂźnĂ©s, mais qui veulent faire entendre une autre voix dans les espaces protestataires comme dans les arĂšnes de la politique instituĂ©e.This article analyses the impact of the 20 February Movement that emerged in the wake of the 2011 Arab uprisings by focusing on the trajectories of militants in the aftermath of the mobilization. Around the first anniversary of the February 20 demonstration, the movementâs abeyance created a feeling of disillusionment among militants. This feeling not only contributed to demobilization, but also to the redeployment of political struggles toward other scales, temporalities, and social spaces. The events of 2011 undeniably marked a generation of militants who are part of the continuity of the struggles of their elders but who also want to make other voices heard in contentious spaces as in the arenas of institutionalized politics
Transgressions étudiantes et conflits de proximité dans le quartier montréalais de Milton-Parc
Le quartier montrĂ©alais de Milton-Parc, parfois appelĂ© « ghetto McGill », est souvent associĂ© au clivage entre deux populations coexistant dans un mĂȘme espace, mais interagissant peu : il y a le Milton-Parc des riverains, composĂ© des mĂ©nages qui y sont installĂ©s de longue date, et le Milton-Parc des Ă©tudiants sans cesse renouvelĂ© par la succession des cohortes universitaires. Cet article revient sur un travail dâobservation et dâentretiens menĂ© dans le quartier en 2019. Face aux conflits de proximitĂ© suscitĂ©s par les pratiques transgressives Ă©tudiantes (en particulier les dĂ©bordements lors des fĂȘtes et lâaccumulation et le mauvais tri des ordures mĂ©nagĂšres), nous proposons que les « normes pratiques » de la rĂ©gulation se caractĂ©risent par une tolĂ©rance relative face aux Ă©carts des Ă©tudiants. Cette tolĂ©rance prend appui sur des reprĂ©sentations morales du quartier perçu comme zone proprement Ă©tudiante justifiant des formes de rĂ©gulation adaptĂ©es aux styles de vie de cette population. MĂȘme sâil peut arriver que les riverains aient recours Ă la loi, cette tolĂ©rance relative et les modes de rĂ©gulation qui en rĂ©sultent reposent sur une tendance Ă lâindulgence pour des pratiques assimilĂ©es Ă la « culture Ă©tudiante » ou Ă la « jeunesse », Ă mĂȘme de susciter des projections intergĂ©nĂ©rationnelles chez les riverains.The Montreal neighbourhood of Milton-Parc, sometimes referred to as the âMcGill Ghetto,â is often associated with a divide between two populations that coexist in the same space while interacting little: there is the Milton-Parc of the local residents, composed of households that have been vested in the area for a long time, and there is the Milton-Parc of the students, continuously renewed by successions of student cohorts. This article draws on observations and interviews conducted in the neighbourhood in 2019. As regards conflicts of proximity caused by transgressive student behaviour (in particular, excessive partying and the accumulation and improper sorting of household waste), we posit that the de facto social norms are characterized by a relative tolerance of student transgressions. This tolerance is based on the moral representations of the neighbourhood as a student zone and, hence, as accepting of the lifestyles of the student population. In other words, although some local residents take recourse to the law when faced with student transgressions, the McGill Ghetto is, overall, characterized by a relative tolerance and its attendant social norms for the practices inherent to âstudent cultureâ or âyouth,â at times even eliciting generational projections among the local residents
DETAILED DESIGN OF A RANKINE VAPOR MICROTURBOPUMP FOR ELEVATED TEMPERATURE OPERATION
Abstract: This paper presents the design of a microturbopump aimed at operation in a Rankine vapor power cycle with superheated steam. Analytical, numerical models and past experience were used to design the various components specifically for elevated temperature operation, including a radial outflow microturbine, a spiral groove viscous micropump, fluid film bearings and seals. Based also on a previous study of heat and stress management, the final layout of a demonstration device was defined, consisting of seven wafers (silicon, glass/quartz, and silicon-on-insulator) that are microfabricated using techniques such as deep reactive ion etching (DRIE), advanced oxide etching, wet etching, anodic and fusion bonding. This device is the first microturbine design that manages heat flows using insulation materials and strategies, which is an important milestone in achieving efficient and robust micro heat engines
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