Autre cause de mort subite du nourrisson: à propos d’un cas clinique de syndrome du QT long congenital

Le syndrome du QT long congénital est une maladie rythmique liée à une mutation génétique et caractérisée par un espace QT allongé sur l'électrocardiogramme, des arythmies malignes type torsade de pointe et  fibrillation ventriculaire entraînant une mort subite. Les gènes impliqués dans ces mutations codent pour des sous unités des canaux ioniques responsables de l'activité électrique cardiaque. Le diagnostic est basé sur l'électrocardiogramme, une enquête familiale et l'étude génétique. Le traitement repose sur les  bêtabloquants, la sympathectomie et le stimulateur cardiaque. Nous rapportons le cas d'un nourrisson de 2 ans retrouvé en état de mort apparente. Nous discutons de sa prise en charge initiale, de l'enquête familiale et de son suivi ultérieur.Key words: Syndrome du QT long, canalopathie rythmique, mort subit

First analysis of a numerical benchmark for 2D columnar solidification of binary alloys

International audienceDuring the solidification of metal alloys, chemical heterogeneities at the product scale (macrosegregation) develop. Numerical simulation tools are beginning to appear in the industry, however their predictive capabilities are still limited. We present a numerical benchmark exercise treating the performance of models in the prediction of macrosegregation. In a first stage we defined a "minimal" (i.e. maximally simplified) solidification model, describing the coupling of the solidification of a binary alloy and of the transport phenomena (heat, solute transport and fluid flow) that lead to macrosegregation in a fully columnar ingot with a fixed solid phase. This model is solved by four different numerical codes, employing different numerical methods (FVM and FEM) and various solution schemes. We compare the predictions of the evolution of macrosegregation in a small (10×6 cm) ingot of Sn-10wt%Pb alloys. Further, we present the sensitivities concerning the prediction of instabilities leading to banded channel mesosegregations

The CORDEX.be initiative as a foundation for climate services in Belgium

The CORDEX.be project created the foundations for Belgian climate services by producing high-resolution Belgian climate information that (a) incorporates the expertise of the different Belgian climate modeling groups and that (b) is consistent with the outcomes of the international CORDEX ("COordinated Regional Climate Downscaling Experiment") project. The key practical tasks for the project were the coordination of activities among different Belgian climate groups, fostering the links to specific international initiatives and the creation of a stakeholder dialogue. Scientifically, the CORDEX.be project contributed to the EURO-CORDEX project, created a small ensemble of High-Resolution (H-Res) future projections over Belgium at convection-permitting resolutions and coupled these to seven Local Impact Models. Several impact studies have been carried out. The project also addressed some aspects of climate change uncertainties. The interactions and feedback from the stakeholder dialogue led to different practical applications at the Belgian national level

CD133 Is a Marker of Bioenergetic Stress in Human Glioma

Mitochondria dysfunction and hypoxic microenvironment are hallmarks of cancer cell biology. Recently, many studies have focused on isolation of brain cancer stem cells using CD133 expression. In this study, we investigated whether CD133 expression is regulated by bioenergetic stresses affecting mitochondrial functions in human glioma cells. First, we determined that hypoxia induced a reversible up-regulation of CD133 expression. Second, mitochondrial dysfunction through pharmacological inhibition of the Electron Transport Chain (ETC) produced an up-regulation of CD133 expression that was inversely correlated with changes in mitochondrial membrane potential. Third, generation of stable glioma cells depleted of mitochondrial DNA showed significant and stable increases in CD133 expression. These glioma cells, termed rho0 or ρ0, are characterized by an exaggerated, uncoupled glycolytic phenotype and by constitutive and stable up-regulation of CD133 through many cell passages. Moreover, these ρ0 cells display the ability to form “tumor spheroids” in serumless medium and are positive for CD133 and the neural progenitor cell marker, nestin. Under differentiating conditions, ρ0 cells expressed multi-lineage properties. Reversibility of CD133 expression was demonstrated by transfering parental mitochondria to ρ0 cells resulting in stable trans-mitochondrial “cybrid” clones. This study provides a novel mechanistic insight about the regulation of CD133 by environmental conditions (hypoxia) and mitochondrial dysfunction (genetic and chemical). Considering these new findings, the concept that CD133 is a marker of brain tumor stem cells may need to be revised

Merkel Cells as Putative Regulatory Cells in Skin Disorders: An In Vitro Study

Merkel cells (MCs) are involved in mechanoreception, but several lines of evidence suggest that they may also participate in skin disorders through the release of neuropeptides and hormones. In addition, MC hyperplasias have been reported in inflammatory skin diseases. However, neither proliferation nor reactions to the epidermal environment have been demonstrated. We established a culture model enriched in swine MCs to analyze their proliferative capability and to discover MC survival factors and modulators of MC neuroendocrine properties. In culture, MCs reacted to bFGF by extending outgrowths. Conversely, neurotrophins failed to induce cell spreading, suggesting that they do not act as a growth factor for MCs. For the first time, we provide evidence of proliferation in culture through Ki-67 immunoreactivity. We also found that MCs reacted to histamine or activation of the proton gated/osmoreceptor TRPV4 by releasing vasoactive intestinal peptide (VIP). Since VIP is involved in many pathophysiological processes, its release suggests a putative regulatory role for MCs in skin disorders. Moreover, in contrast to mechanotransduction, neuropeptide exocytosis was Ca2+-independent, as inhibition of Ca2+ channels or culture in the absence of Ca2+ failed to decrease the amount of VIP released. We conclude that neuropeptide release and neurotransmitter exocytosis may be two distinct pathways that are differentially regulated

Cardiovascular Rehabilitation

peer reviewedIn industrialized countries, coronary artery disease is the leading cause of mortality. Cardiovascular rehabilitation is one of the most important components of secondary prevention and is mainly prescribed after acute myocardial infarction or coronary artery revascularization. The aims of rehabilitation are not only the improvement of functional capacity but also the correction of risk factors and assistance to social and professional reintegration. There are numerous indications, including not only patients with coronary artery disease but also patients with heart failure and those who underwent cardiac surgery or heart transplantation. Cardiac rehabilitation consists in 3 successive stages. The first is performed during hospital stay. The two others take place in an approved center of multidisciplinary cardiac rehabilitation. The intensity of exercise is determined for each individual patient from the results of stress testing. The beneficial effects are well established: reduction in morbidity and mortality, improvement of quality of life and of pulmonary, cardiac, muscular and metabolic parameters. Its efficacy is only maximal when patients continue performing exercise regularly

Numerical simulation of macrosegregation and formation of channel segregates during alloy solidification using high-order TVD and WENO schemes

International audienceThis paper aims to tackle the problem of thermo‐solutal convection and macrosegregation during ingot solidification of metal alloys. Complex flow structures associated with the development of channels segregate and sharp gradients in the solutal field call for the implementation of accurate methods for numerical modeling of alloy solidification. In particular, the solute transport equation is convection dominated and requires special non‐oscillarity type high‐order schemes to handle the regions of channels segregates

Autre cause de mort subite du nourrisson: À propos d’un cas clinique de syndrome du QT long congénital

SCOPUS: ar.jSCOPUS: ar.jinfo:eu-repo/semantics/publishe

Autre cause de mort subite du nourrisson: À propos d’un cas clinique de syndrome du QT long congénital

SCOPUS: ar.jSCOPUS: ar.jinfo:eu-repo/semantics/publishe