AGENTE MOTORIZADO DON PEDRO SOSA EN EL LÓPEZ SOCAS [Material gráfico]

Copia digital. Madrid : Ministerio de Educación, Cultura y Deporte. Subdirección General de Coordinación Bibliotecaria, 201

Modélisation de structures voiles en béton armé soumises à du cisaillement

Within the French National Program CEOS.fr reinforced concrete shear walls were tested in order to create a database and thereby compare and improve the numerical tools to characterize cracks under severe shear stresses. In this article we present the numerical simulation of the specimen 3 of the program using a stress plane model and two different constitutive laws: the Mazars and the Damage_TC models. Although these laws are extensively validated in traction-compression conditions, it is necessary to evaluate their performances under important shear stresses.Dans le cadre du programme CEOS.fr, des campagnes expérimentales sur des voiles en béton armé soumis à des chargements statiques sévères ont établi une importante base de données permettant la calibration et l'amélioration des outils numériques pour caractériser l'ouverture de fissures sous l'effet de contraintes de cisaillement. L'étude numérique présentée dans ce rapport consiste à reproduire l'essai du voile 3 grâce à une modélisation élément fini 2D en contraintes planes en étudiant la performance de deux lois d'endommagement du code Cast3M : La loi Mazars et la loi Damage_TC. Si ces lois sont extensivement validées dans le passé dans des cas de modèles soumis à des contraintes de traction-compression, il convient de les évaluer sur des structures soumises à de fortes contraintes en cisaillement

Behavior of unrepaired perimembranous ventricular septal defect in young adults

The number of adolescents and young adults with congenital heart defects, including ventricular septal defect (VSD), increases continuously. We evaluated the mid-term outcome of small and unclosed perimembranous VSDs (pmVSDs). All patients with a known unrepaired pmVSD at 16 years of age were selected from our database. The clinical, electrocardiographic, and echocardiographic changes between baseline and the latest follow-up examination were compared. A total of 220 patients (119 males, median age 18 years, interquartile range 7) could be included. During a median follow-up of 6 years (interquartile range 4, range 38), 2 patients died (1%; 1 from sudden death and 1 from end-stage heart failure). Endocarditis occurred in 8 patients (4%). One patient required pacemaker implantation (0.5%) and one required implantable cardioverter-defibrillator implantation (1%). Fifteen patients (7%) required a closing procedure. In 8 patients (4%), the pmVSD closed spontaneously. In the remaining 203 patients (93%), the QRS morphology changed in 5% and 1% lost sinus rhythm (p = 0.0001 and p = 0.015, respectively). The left ventricular ejection fraction and stroke volume index increased from 62 + or - 7% to 67 + or - 8% and from 41 + or - 11 to 44 + or - 15 ml/m(2) (p = 0.0001 and p = 0.035, respectively), the end-systolic diameter decreased, and the end-diastolic diameter did not change. Finally, patients with an open pmVSD developed more pulmonary arterial hypertension during follow-up (from 3% to 9%, p = 0.002). In conclusion, mid-term follow-up of adolescents and young adults with a small and unrepaired pmVSD was not uneventful. Some patients required intervention, but in others, spontaneous closure occurred. Electrocardiographic and structural changes were noticed, for which the clinical significance needs to be determined.status: publishe

Mutational, functional, and expression studies of the TCF4 gene in Pitt-Hopkins syndrome

International audiencePitt-Hopkins syndrome is a severe congenital encephalopathy recently ascribed to de novo heterozygous TCF4 gene mutations. We report a series of 13 novel PHS cases with a TCF4 mutation and show that EEG, brain magnetic resonance imagain (MRI), and immunological investigations provide valuable additional clues to the diagnosis. We confirm a mutational hot spot in the basic domain of the E-protein. Functional studies illustrate that heterodimerisation of mutant TCF4 proteins with a tissue-specific transcription factor is less effective than that homodimerisation in a luciferase reporter assay. We also show that the TCF4 expression pattern in human embryonic development is widespread but not ubiquitous. In summary, we further delineate an underdiagnosed mental retardation syndrome, highlighting TCF4 function during development and facilitating diagnosis within the first year of life