23 research outputs found

    Impaired respiratory burst contributes to infections in PKCδ-deficient patients

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    Patients with autosomal recessive protein kinase C δ (PKCδ) deficiency suffer from childhood-onset autoimmunity, including systemic lupus erythematosus. They also suffer from recurrent infections that overlap with those seen in patients with chronic granulomatous disease (CGD), a disease caused by defects of the phagocyte NADPH oxidase and a lack of reactive oxygen species (ROS) production. We studied an international cohort of 17 PKCδ-deficient patients and found that their EBV-B cells and monocyte-derived phagocytes produced only small amounts of ROS and did not phosphorylate p40phox normally after PMA or opsonized Staphylococcus aureus stimulation. Moreover, the patients' circulating phagocytes displayed abnormally low levels of ROS production and markedly reduced neutrophil extracellular trap formation, altogether suggesting a role for PKCδ in activation of the NADPH oxidase complex. Our findings thus show that patients with PKCδ deficiency have impaired NADPH oxidase activity in various myeloid subsets, which may contribute to their CGD-like infectious phenotype

    Autoantibodies against type I IFNs in patients with life-threatening COVID-19

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    Interindividual clinical variability in the course of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is vast. We report that at least 101 of 987 patients with life-threatening coronavirus disease 2019 (COVID-19) pneumonia had neutralizing immunoglobulin G (IgG) autoantibodies (auto-Abs) against interferon-w (IFN-w) (13 patients), against the 13 types of IFN-a (36), or against both (52) at the onset of critical disease; a few also had auto-Abs against the other three type I IFNs. The auto-Abs neutralize the ability of the corresponding type I IFNs to block SARS-CoV-2 infection in vitro. These auto-Abs were not found in 663 individuals with asymptomatic or mild SARS-CoV-2 infection and were present in only 4 of 1227 healthy individuals. Patients with auto-Abs were aged 25 to 87 years and 95 of the 101 were men. A B cell autoimmune phenocopy of inborn errors of type I IFN immunity accounts for life-threatening COVID-19 pneumonia in at least 2.6% of women and 12.5% of men

    Accidentologie des deux-roues motorisés : Vers une meilleure prise en compte de leur diversité. Rapport Introductif (R0.1)

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    Rapport de rechercheCette étude a pour objectif d'appréhender et mieux comprendre les interactions de facteurs et les mécanismes en jeu dans les accidents des deux-roues motorisés. Elle vise également à mettre en évidence la diversité de ces accidents de façon à permettre la définition de mesures plus adaptée à chaque problème en jeu. Les deux-roues motorisés se distinguent des autres modes de transport terrestres par des différences d'ordre dynamique, perceptif, mais aussi comportemental, attitudinal et social. Parmi les particularités qui les caractérisent, on développera les points suivants : Une population d'usager qui s'accroît de manière continue ; des usagers dont les facteurs socio-démographiques sont de plus en plus hétérogènes, Une offre de véhicules croissante avec une grande diversité de cylindrées, de types etc. ; Un comportement dynamique et une conduite spécifiques en fonction du type de véhicule et de ses capacités ; Une place à part au sein du trafic ; Une accidentalité particulière : grande vulnérabilité des usagers, inadéquations entre les deux-roues motorisés et le système de circulation, les interactions. Partenariat : INRETS-MA, LAB-GIE PSA/Renaul

    Accidentologie des deux-roues motorisés : Vers une meilleure prise en compte de leur diversité. Rapport Introductif (R0.1)

    No full text
    Rapport de rechercheCette étude a pour objectif d'appréhender et mieux comprendre les interactions de facteurs et les mécanismes en jeu dans les accidents des deux-roues motorisés. Elle vise également à mettre en évidence la diversité de ces accidents de façon à permettre la définition de mesures plus adaptée à chaque problème en jeu. Les deux-roues motorisés se distinguent des autres modes de transport terrestres par des différences d'ordre dynamique, perceptif, mais aussi comportemental, attitudinal et social. Parmi les particularités qui les caractérisent, on développera les points suivants : Une population d'usager qui s'accroît de manière continue ; des usagers dont les facteurs socio-démographiques sont de plus en plus hétérogènes, Une offre de véhicules croissante avec une grande diversité de cylindrées, de types etc. ; Un comportement dynamique et une conduite spécifiques en fonction du type de véhicule et de ses capacités ; Une place à part au sein du trafic ; Une accidentalité particulière : grande vulnérabilité des usagers, inadéquations entre les deux-roues motorisés et le système de circulation, les interactions. Partenariat : INRETS-MA, LAB-GIE PSA/Renaul

    EXPERIMENTAL ASSESSMENT OF THE QUANERGY M8 LIDAR SENSOR

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    In this paper, some experiments with the Quanergy M8 scanning LIDAR system are related. The distance measurement obtained with the Quanergy M8 can be influenced by different factors. Moreover, measurement errors can originate from different sources. The environment in which the measurements are performed has an influence (temperature, light, humidity, etc.). Errors can also arise from the system itself. Then, it is necessary to determine the influence of these parameters on the quality of the distance measurements. For this purpose different studies are presented and analyzed. First, we studied the temporal stability of the sensor by analyzing observations during time. Secondly, the assessment of the distance measurement quality has been conducted. The aim of this step is to detect systematic errors in measurements regarding the range. Differents series of measurements have been conducted : at different range and in diffrent conditions (indoor and outdoor). Finally, we studied the consistency between the differents beam of the LIDAR

    EXPERIMENTAL ASSESSMENT OF THE QUANERGY M8 LIDAR SENSOR

    Full text link
    In this paper, some experiments with the Quanergy M8 scanning LIDAR system are related. The distance measurement obtained with the Quanergy M8 can be influenced by different factors. Moreover, measurement errors can originate from different sources. The environment in which the measurements are performed has an influence (temperature, light, humidity, etc.). Errors can also arise from the system itself. Then, it is necessary to determine the influence of these parameters on the quality of the distance measurements. For this purpose different studies are presented and analyzed. First, we studied the temporal stability of the sensor by analyzing observations during time. Secondly, the assessment of the distance measurement quality has been conducted. The aim of this step is to detect systematic errors in measurements regarding the range. Differents series of measurements have been conducted : at different range and in diffrent conditions (indoor and outdoor). Finally, we studied the consistency between the differents beam of the LIDAR.</jats:p

    Analyse comparative de procédures d'accidents mortels et non mortels. Rapport scientifique Tâche 3

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    Rapport de rechercheCe projet a pour ambition d'identifier les différentes facettes de l'insécurité des 2RM : du point de vue de l'usager 2RM, du point de vue des autres usagers et du point de vue de l'infrastructure routière. Il s'appuie sur trois approches complémentaires : L'analyse des accidents de deux-roues à partir de données EDA (Etudes Détaillées d'Accidents) et d'exploitations de procédures (Procès-Verbaux), L'analyse comportementale des situations de conduite en deux-roues en interaction avec les autres usagers, L'analyse des représentations réciproques des usagers de deux-roues et des autres usagers. Ce rapport fait état des travaux réalisés dans le cadre de la Tâche 3 : Analyse comparative de procédures d'accidents mortels et non mortels. Cette étude accidentologique "quantitative approfondie" à partir de procédures d'accidents est complémentaire de l'analyse "clinique" des EDA (Tâche 1). L'objectif est d'établir une distinction entre les accidents mortels et non mortels selon plusieurs paramètres : le type de situation d'accident, le type de véhicule impliqué, les sources de dysfonctionnements, etc., et de rendre compte des configurations accidentelles les plus récurrente

    Analysis of accident data for test scenario definition in the ASSESS project

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    The overall purpose of the ASSESS project is to develop a relevant and standardised set of test and assessment methods and associated tools for integrated vehicle safety systems, primarily focussing on currently available pre-crash sensing systems. The first stage of the project was to define casualty relevant accident scenarios so that the test scenarios will be developed based on accident scenarios which currently result in the greatest injury outcome, measured by a combination of casualty severity and casualty frequency. The first analysis stage was completed using data from a range of accident databases, including those which were nationally representative (STATS19, UK and STRADA, SE) and in-depth sources which provided more detailed parameters to characterise the accident scenarios (GIDAS, DE and OTS, UK). A common analysis method was developed in order to compare the data from these different sources, and while the data sets were not completely compatible, the majority of the data was aligned in such a way that allowed a useful comparison to be made. As the ASSESS project focuses on pre-crash sensing systems fitted to passenger cars, the data selected for the analysis was "injury accidents which involved at least one passenger car". The accident data analysis yielded the following ranked list of most relevant accident scenarios: Rank Accident scenario 1 Driving accident - single vehicle loss of control 2 Accidents in longitudinal traffic (same and opposite directions) 3 Accidents with turning vehicle(s) or crossing paths in junctions 4 Accidents involving pedestrians The ranked list highlights the relatively large role played by "accidents in longitudinal traffic", and "accidents with turning vehicle(s) or crossing paths in junctions" (the second and third most prevalent accident scenarios, respectively). The pre-crash systems addressed in ASSESS propose to yield beneficial safety outcomes with specific regard to these accident scenarios. This indicates that the ASSESS project is highly relevant to the current casualty crash problem. In the second stage of the analysis a selection of these accident scenarios were analysed further to define the accident parameters at a more detailed level .This paper describes the analysis approach and results from the first analysis stage
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