Bibliothèque Nationale de France

Background Myosin heavy chain 7 ( MYH7 )-related myopathies ( MYH7 -RMs) are a group of muscle disorders linked to pathogenic variants in the MYH7 gene, encoding the slow/beta-cardiac myosin heavy chain, which is highly expressed in skeletal muscle and heart. The phenotype is heterogeneous including distal, predominantly axial or scapuloperoneal myopathies with variable cardiac involvement. Methods We retrospectively analysed the clinical, muscle MRI, genetic and myopathological features of 57 MYH7 patients. Patients received a thorough neurological (n=57, 100%), cardiac (n=51, 89%) and respiratory (n=45, 79%) assessment. Muscle imaging findings and muscle biopsies were reappraised in 19 (33%) and 27 (47%) patients, respectively. Results We identified three phenotypes with varying degrees of overlap: distal myopathy (70%), scapuloperoneal (23%) and axial with peculiar cervical spine rigidity called the ‘sphinx’ phenotype (7%). 14% of patients had either dilated cardiomyopathy, hypertrophic cardiomyopathy or left ventricular non-compaction cardiomyopathy. 31% of patients had prominent respiratory involvement, including all patients with the ‘sphinx’ phenotype. Muscle MRI showed involvement of tibialis anterior, followed by quadriceps, and erector spinae in patients with axial phenotype. Cores represented the most common myopathological lesion. We report 26 pathogenic variants of MYH7 gene, 9 of which are novel. Conclusions MYH7 -RMs have a large phenotypic spectrum, including distal, scapuloperoneal or axial weakness, and variable cardiac and respiratory involvement. Tibialis anterior is constantly and precociously affected both clinically and on muscle imaging. Cores represent the most common myopathological lesion. Our detailed description of MYH7 -RMs should improve their recognition and management.

In the last decade, the hyperspectral imaging (HSI) method allowed performing non-invasive analysis in the field of cultural heritage. However, a considerable limitation was given by redundant and time-consuming features, with the necessary application of statistical algorithms and image-processing tools to extract relevant information. In this study, the Centro Nacional de Aceleradores (CNA) and the Group of Interdisciplinary Physics (GFI) of the School of Engineering (ETSI) of the University of Seville tested the application of three different hyperspectral cameras in the visible and near-infrared (VNIR) and short-wave infrared (SWIR) range for the investigation of an ancient painting. A reference-based procedure was realised to build a starting personal library and to evaluate the best working conditions for non-invasive and non-destructive characterisation with data treatment using the commercially available software Evince® and Specim IQ® to apply, respectively, the Principal Component Analysis (PCA) model functions and the classification method. The evaluation of the protocol was tested by acquiring complementary information by X-ray fluorescence (XRF), Ultraviolet Luminescence (UVL) imaging, and Infrared Reflectography (IRR). This exploration established a simplified protocol to analyse the large collection of paintings of the Archbishop’s Palace and the Cathedral of Seville.

X-ray computed tomography (XRCT) imaging allows non-destructive visualization of the structure of various materials. Applied to wooden objects, it allows determination of their morphologies or manufacturing techniques, but also measurement of growth ring widths. We have applied XRCT to a selection of 38 mummy labels. This funerary furniture, made up of endemic or imported tree species, has survived thanks to environmental conditions in very large quantities in regions in Middle and Upper Egypt and is featured now in museum collections across the globe. Mummy labels thus represent a unique and abundant data source to build floating or absolutely dated dendrochronological chronologies for this period. Here we discuss the possible contributions and limitations of XRCT for the analysis of these artifacts and show that the approach allows identification of discriminating markers for the identification of certain species on the transverse plane, but that the insufficient resolution of the tangential and radial planes normally prevents formal identification of species. By contrast, XRCT undeniably enhances the visibility of toolmarks (in terms of numbers and depth), and thereby allows highlighting marks that remain invisible to the naked eye; XRCT also provides key insights into cutting methods and the calibers used and yields new information on silvicultural practices and the knowhow of Egyptian craftsmen. Finally, the measurement of ring widths on XRCT imagery is also more accurate than what can be achieved by traditional dendrochronological measurements, especially in the case of cuts realized on a slab. The approach also confirms the limited potential of local broadleaved species for dendrochronological approaches due to unreadable or poorly visible tree rings and mostly short tree-ring sequences.

Recent years witnessed an increase in attention to femicide, or the killing of women because of their gender. Prior empirical studies have drawn attention to the prevalence of female homicide victimization, but most have been unable to give a detailed overview of the specific contexts in which women are killed, and to what extent female homicide victimization is unique, i.e., in that it differs from male victimization. This exploratory study aims, first, to map the nature and scope of female homicide in six European countries: Denmark, Finland, France, the Netherlands, Sweden and Switzerland; and, secondly, to compare female homicide victimization with male homicide victimization. Using data from a new uniform homicide recording system, the European Homicide Monitor, this study allowed for unique cross-country comparisons and a detailed breakdown by victim-offender relationship, and type of homicide. Results indicated that female homicide victimization rates remained relatively stable during the last decade, with a narrowing gender gap. Furthermore, results reflected substantial heterogeneity in the context in which women were victimized. Finally, female homicide victimization differs in many respects from male victimization, but overlaps do exist. Such heterogeneity, and possible overlaps with male victimization call for differential approaches.

The present research aimed to explore whether negative stereotypes and domain identification moderate novice participants’ performance on a soccer-dribbling task, using a novel statistical procedure to explore within-subjects moderation effects. Fifty-four women with no soccer experience (Mage = 19.92, SDage = 1.0) were recruited and assigned to a negative stereotype condition or a nullified-stereotype condition and performed a soccer-dribbling task as fast as possible. Using moderation analyses for repeated measures, results showed (i) that inducing a negative stereotype did not moderate participants’ performance, and (ii) that domain identification did not moderate participants’ performance in a stereotype threat situation. In conclusion, the novice participants performance did not depend on the value of domain identification and the stereotype condition. More research is nonetheless needed to disentangle the question of how and why novice participants can be impacted by a stereotype threat situation.

A part of European culture since the 18th century, the “History of the Ottoman Empire” of Dimitrie Cantemir calls for a new reading, especially since the erudite historian Virgil Cândea discovered its original manuscript, which scholars have long sought. Beyond the historiographical value of the work as documentation and critical wit, its clear and flowing “story-like” writing illustrates the indissoluble link between history and storytelling mentioned by Paul Ricœur. It is easier to understand why, thanks to this condition, it did not pose too complicated problems of translation, easing the effort of translators and offering itself to 18th-century (and later) readers as an instructive and enjoyable read. The criticism came especially from the angle of scholarship with the development of the critical spirit in historiography in the 19th century and the formation of new generations of scholars specializing in Ottoman studies. However, while Cantemir’s history of the Ottomans has given way to other later works as documentation and analysis, it retains the same interest as a narrative discourse, the mark of time contributing no less to its fascination.

La pandémie de COVID-19 a forcé les bibliothèques parlementaires à s’adapter pour continuer à remplir leur rôle auprès des décideurs publics. La Bibliothèque de l’Assemblée nationale du Québec a pris une grande importance dans la gestion de crise par son intégration à quatre cellules de veille stratégique mises en place par l’administration parlementaire pour filtrer et synthétiser les informations concernant la COVID-19. Ces cellules avaient pour objectif d’aider le comité directeur de l’Assemblée nationale à prendre des décisions éclairées. Cet article présente l’expérience de la Bibliothèque de l’Assemblée nationale au sein des cellules de veille stratégiques et souligne l’importance des bibliothèques parlementaires pour fournir des informations fiables et pertinentes aux décideurs institutionnels en période de crise.

Les bibliothèques parlementaires sont confrontées à un changement périodique de leurs clientèles politiques en fonction du cycle électoral. L’accueil d’une nouvelle législature est par conséquent un événement crucial pour ces institutions ; il est alors essentiel de promouvoir leurs services pour montrer leur utilité aux nouveaux parlementaires. Qui plus est, les parlementaires ne sont souvent pas conscients de l’étendue des services offerts par les bibliothèques parlementaires, dont l’offre de services diffère des milieux documentaires communs. Cet article présente un aperçu des initiatives entreprises par les bibliothèques parlementaires canadiennes lors de l’accueil d’une nouvelle législature, ainsi que des activités de promotion utilisées pour faire connaître leurs services à la nouvelle députation et au nouveau personnel politique.

Plus de trois ans après la crise sanitaire, des évolutions profondes ont eu lieu dans les milieux professionnels, quel que soit le secteur d’activité, et quel que soit le cadre, public ou privé. Les changements et transformations ont été plus subis que choisis, et certains retours en arrière ne sont plus envisageables. Le présent article veut explorer les types de changements et de transformation, en général et dans l’activité des milieux documentaires, et les appréhender dans leurs conséquences sur le travail, les collectifs de travail et les individus. La grille d’analyse des risques psychosociaux (RPS), permet toutefois d’envisager une trajectoire qui intégrerait la qualité de vie au travail, via le bien-être psychologique. Enfin, les conséquences pour les gestionnaires et encadrants (pour la fonction publique) seront identifiées. En conclusion, on envisagera de quoi les RPS peuvent être le signe.

Abstract: Many sources of error in hydroclimatic data can affect hydrological modelling; yet the impact of streamflow data quality is poorly quantified. This work aims to investigate whether inconsistencies found in streamflow time series commonly available for hydrological studies (typically in national streamflow archives) have an impact on the efficiency and the parameter estimates of rainfall–runoff models. Hydroclimatic data were gathered at the hourly time step over the period 1998–2018 for a set of 30 catchments in France. Hydrological modelling was carried out with the lumped conceptual GR5H model. A typology of ‘realistic’ suspicious streamflow was established to set up several error models in order to corrupt the data. Our results suggest that common suspicious streamflow data do not have a strong impact on model efficiency and parameter estimates overall, but may still be an important source of instability and lack of robustness when working on a single catchment.

Background: Short-term forecasts of infectious disease contribute to situational awareness and capacity planning. Based on best practice in other fields and recent insights in infectious disease epidemiology, one can maximise forecasts’ predictive performance by combining independent models into an ensemble. Here we report the performance of ensemble predictions of COVID-19 cases and deaths across Europe from March 2021 to March 2022. Methods: We created the European COVID-19 Forecast Hub, an online open-access platform where modellers upload weekly forecasts for 32 countries with results publicly visualised and evaluated. We created a weekly ensemble forecast from the equally-weighted average across individual models' predictive quantiles. We measured forecast accuracy using a baseline and relative Weighted Interval Score (rWIS). We retrospectively explored ensemble methods, including weighting by past performance. Results: We collected weekly forecasts from 48 models, of which we evaluated 29 models alongside the ensemble model. The ensemble had a consistently strong performance across countries over time, performing better on rWIS than 91% of forecasts for deaths (N=763 predictions from 20 models), and 83% forecasts for cases (N=886 predictions from 23 models). Performance remained stable over a 4-week horizon for death forecasts but declined with longer horizons for cases. Among ensemble methods, the most influential choice came from using a median average instead of the mean, regardless of weighting component models. Conclusions: Our results support combining independent models into an ensemble forecast to improve epidemiological predictions, and suggest that median averages yield better performance than methods based on means. We highlight that forecast consumers should place more weight on incident death forecasts than case forecasts at horizons greater than two weeks. Funding: European Commission, Ministerio de Ciencia, Innovación y Universidades, FEDER; Agència de Qualitat i Avaluació Sanitàries de Catalunya; Netzwerk Universitätsmedizin; Health Protection Research Unit; Wellcome Trust; European Centre for Disease Prevention and Control; Ministry of Science and Higher Education of Poland; Federal Ministry of Education and Research; Los Alamos National Laboratory; German Free State of Saxony; NCBiR; FISR 2020 Covid-19 I Fase; Spanish Ministry of Health / REACT-UE (FEDER); National Institutes of General Medical Sciences; Ministerio de Sanidad/ISCIII; PERISCOPE European H2020; PERISCOPE European H2021; InPresa; National Institutes of Health, NSF, US Centers for Disease Control and Prevention, Google, University of Virginia, Defense Threat Reduction Agency.