108 research outputs found

    Intégrer des ressources numériques dans les collections

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    Cet ouvrage s’inscrit dans la continuitĂ© de plusieurs autres volumes de la collection afin de proposer aux bibliothĂ©caires et documentalistes des clĂ©s pour aborder et gĂ©rer efficacement la documentation dĂ©matĂ©rialisĂ©e : une douzaine d’auteurs s’attachent Ă  prĂ©senter les modalitĂ©s de sĂ©lection, d’acquisition, de signalement, d’évaluation et de conservation propres Ă  ces ressources en dĂ©veloppant en filigrane un questionnement sur la fonction de la bibliothĂšque. Prenant appui aussi bien sur des bibliothĂšques de lecture publique que des universitĂ©s, le plan s’organise autour de quatre parties pragmatiques et opĂ©ratoires : connaĂźtre le contexte ; sĂ©lectionner et acquĂ©rir ; intĂ©grer et sĂ©lectionner et, enfin, Ă©valuer et conserver

    In Antisynthetase Syndrome, ACPA Are Associated With Severe and Erosive Arthritis: An Overlapping Rheumatoid Arthritis and Antisynthetase Syndrome

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    International audienceAbstract: Anticitrullinated peptide/protein antibodies (ACPA), which are highly specific for rheumatoid arthritis (RA), may be found in some patients with other systemic autoimmune diseases. The clinical significance of ACPA in patients with antisynthetase syndrome (ASS), a systemic disease characterized by the association of myositis, interstitial lung disease, polyarthralgia, and/or polyarthritis, has not yet been evaluated with regard to phenotype, prognosis, and response to treatment. ACPA-positive ASS patients were first identified among a French multicenter registry of patients with ASS. Additionally, all French rheumatology and internal medicine practitioners registered on the Club Rhumatismes et Inflammation web site were asked to report their observations of ASS patients with ACPA. The 17 collected patients were retrospectively studied using a standardized questionnaire and compared with 34 unselected ACPA-negative ASS patients in a case–control study. All ACPA-positive ASS patients suffered from arthritis versus 41% in the control group (P 7-year mean follow-up, extra-articular outcomes and survival were not different. ACPA-positive ASS patients showed an overlapping RA–ASS syndrome, were at high risk of refractory erosive arthritis, and might experience ASS flare when treated with antitumor necrosis factor drugs. In contrast, other biologics such as anti-CD20 mAb were effective in this context, without worsening systemic involvements

    Pathogenic variants in THSD4, encoding the ADAMTS-like 6 protein, predispose to inherited thoracic aortic aneurysm

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    Purpose Thoracic aortic aneurysm and dissection (TAAD) is a life-threatening disease with often unrecognized inherited forms. We sought to identify novel pathogenic variants associated with autosomal dominant inheritance of TAAD. Methods We analyzed exome sequencing data from 35 French TAAD families and performed next-generation sequencing capture panel of genes in 1114 unrelated TAAD patients. Functional effects of pathogenic variants identified were validated in cell, tissue, and mouse models. Results We identified five functional variants inTHSD4of which two heterozygous variants lead to a premature termination codon.THSD4encodes ADAMTSL6 (member of the ADAMTS/L superfamily), a microfibril-associated protein that promotes fibrillin-1 matrix assembly. TheTHSD4variants studied lead to haploinsufficiency or impaired assembly of fibrillin-1 microfibrils.Thsd4(+/-)mice showed progressive dilation of the thoracic aorta. Histologic examination of aortic samples from a patient carrying aTHSD4variant and fromThsd4(+/-)mice, revealed typical medial degeneration and diffuse disruption of extracellular matrix. Conclusion These findings highlight the role of ADAMTSL6 in aortic physiology and TAAD pathogenesis. They will improve TAAD management and help develop new targeted therapies

    Détermination de la polarisation des gluons dans le nucléon par la production de hadrons à grande impulsion transverse à COMPASS

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    L objectif principal de l expĂ©rience COMPASS au CERN est la dĂ©termination de la polarisation des gluons dans le nuclĂ©on, Delta G / G. On mesure pour cela l asymĂ©trie d hĂ©licitĂ© du processus de fusion photon gluon, dans la diffusion de muons polarisĂ©s sur une cible de deutĂ©rons polarisĂ©s. Ce processus peut ĂȘtre sĂ©lectionnĂ© par la production de hadrons Ă  grande impulsion transverse (p_T), ce qui permet de disposer d une grande statistique. En contrepartie, la prĂ©sence d un bruit de fond physique complique l extraction de Delta G / G. Cette thĂšse prĂ©sente diffĂ©rentes Ă©tudes menĂ©es afin d optimiser la dĂ©termination de Delta G / G dans ce canal. Une Ă©tude sur l alignement du spectromĂštre est notamment prĂ©sentĂ©e, amĂ©liorant ainsi sa rĂ©solution. Les performances des 12 dĂ©tecteurs de type Micromegas ont Ă©galement Ă©tĂ© Ă©tudiĂ©es au cours du run 2004. Puis, les asymĂ©tries obtenues Ă  partir de l analyse des donnĂ©es 2002 Ă  2004 sont calculĂ©es, pour diffĂ©rentes variantes de la sĂ©lection Ă  grand p_T: 1 ou 2 hadrons, petit ou grand Q^2. Une optimisation de la sĂ©lection a aussi Ă©tĂ© dĂ©veloppĂ©e Ă  l aide d un rĂ©seau de neurones. Une Ă©tude dĂ©taillĂ©e de la fausse asymĂ©trie expĂ©rimentale est Ă©galement rĂ©alisĂ©e. L extraction de Delta G / G est ensuite dĂ©crite, Ă  partir de simulations Monte Carlo (utilisant PYTHIA ou LEPTO). Pour la premiĂšre fois, l asymĂ©trie des processus dits photons rĂ©solus est estimĂ©e. Une amĂ©lioration de la reconstruction de la fraction d impulsion du nuclĂ©on portĂ©e par le gluon est Ă©galement proposĂ©e, par la reconstruction de pseudo-jets. Enfin, les petites valeurs obtenues pour Delta G / G sont discutĂ©es, dans le cadre de la dĂ©composition en spin du nuclĂ©on.The main goal of the COMPASS experiment at CERN is the determination of the gluon polarisation in the nucleon, Delta G / G. For this, the helicity asymmetry of the photon gluon fusion process is measured, in the scattering of polarized muons on a polarised deuteron target. This process can be tagged by the production of hadrons with high transverse momentum (p_T), that allows to get a large statistics. On the other hand, a physical background remains and complicates Delta G / G extraction. This PhD thesis presents different studies performed to optimize the determination of Delta G / G in this channel. In particular, a study of the spectrometer alignment is presented, leading to an improvement of its resolution. Performances of the 12 Micromegas detectors have also been studied during 2004 run. Then, the asymmetries obtained in the analysis of 2002 to 2004 data are calculated, for various high p_T selection: 1 or 2 hadrons, low or high Q^2. An optimization of the selection, based on a neural network, has also been developped.,and a detailed study of the experimental false asymmetry has been performed. Delta G / G extraction is then described, based on Monte Carlo simulations (using PYTHIA or LEPTO). For the first time, the asymmetry of so called resolved photon processes is estimated. An improvement on the reconstruction of nucleon momentum fraction carried by the gluon is also proposed, by reconstructing pseudo-jets. Finally, small values obtained for Delta G / G are discussed, within the framework of the nucleon spin decomposition.ORSAY-PARIS 11-BU Sciences (914712101) / SudocSudocFranceF

    An updated checklist of mosquito species (Diptera: Culicidae) from Madagascar

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    An updated checklist of 235 mosquito species from Madagascar is presented. The number of species has increased considerably compared to previous checklists, particularly the last published in 2003 (178 species). This annotated checklist provides concise information on endemism, taxonomic position, developmental stages, larval habitats, distribution, behavior, and vector-borne diseases potentially transmitted. The 235 species belong to 14 genera: Aedeomyia (3 species), Aedes (35 species), Anopheles (26 species), Coquillettidia (3 species), Culex (at least 50 species), Eretmapodites (4 species), Ficalbia (2 species), Hodgesia (at least one species), Lutzia (one species), Mansonia (2 species), Mimomyia (22 species), Orthopodomyia (8 species), Toxorhynchites (6 species), and Uranotaenia (73 species). Due to non-deciphered species complexes, several species remain undescribed. The main remarkable characteristic of Malagasy mosquito fauna is the high biodiversity with 138 endemic species (59%). Presence and abundance of species, and their association, in a given location could be a bio-indicator of environmental particularities such as urban, rural, forested, deforested, and mountainous habitats. Finally, taking into account that Malagasy culicidian fauna includes 64 species (27%) with a known medical or veterinary interest in the world, knowledge of their biology and host preference summarized in this paper improves understanding of their involvement in pathogen transmission in Madagascar
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