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3. Mammals part 2

Author

Eppley, T. M, primary, Steffens, K. J. E., additional, Colquhoun, I. C., additional, Birkinshaw, C. R., additional, Samonds, K. E, additional, Goodman, S. M., additional, Alumbaugh, J. L., additional, Simmons, N. B, additional, Veron, G., additional, Michaud, M., additional, Jansa, S. A., additional, Carleton, M. D., additional, Ramasindrazana, B., additional, Randriamoria, T. M, additional, Rahelinirina, S., additional, Duchemin, J.-B, additional, Duplantier, J.-M., additional, Soarimalala, V., additional, Jenkins, P. D, additional, Olson, L. E, additional, Everson, K. M, additional, Nicoll, M. E, additional, Schoeman, M. C., additional, Andrianaivoarivelo, R., additional, Andriafidison, D., additional, Razafimanahaka, J. H., additional, Razafindrakoto, N., additional, Oleksy, R. Z., additional, Racey, P. A., additional, Ralisata, M., additional, Kofoky, A. F., additional, Schliemann, H., additional, Blanco, M. B, additional, Kappeler, P. M, additional, Radespiel, U., additional, Rasoloarison, R. M., additional, Salmona, J., additional, Yoder, A. D., additional, Rasoloharijaona, S., additional, and Louis, E. E., additional

Published
2022
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7. Évaluation de l’efficacité du traitement préexposition par l’Hydroxychloroquine sur le risque de l’infection par le Sars-cov.2 et de l’efficacité de la vaccination anti-COVID au cours du lupus et/ou du Gougerot Sjögren : essai multicentrique Prepcov

Author

Alric, L., primary, Stéphanie, F., additional, Berard, E., additional, Lebray, P., additional, Viallard, J.F., additional, Chauveau, D., additional, Sailler, L., additional, Michaud, M., additional, Pugnet, G., additional, Migueres, M., additional, and Cacoub, P., additional

Published
2024
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11. Risk factors for bleeding, including platelet count threshold, in newly diagnosed immune thrombocytopenia adults

Author

Piel‐Julian, M.‐L., Mahévas, M., Germain, J., Languille, L., Comont, T., Lapeyre‐Mestre, M., Payrastre, B., Beyne‐Rauzy, O., Michel, M., Godeau, B., Adoue, D., Moulis, G., Alric, L., Arista, S., Astudillo, L., Balardy, L., Betrian, S., Bonnet, D., Borel, C., Brechemier, D., Brun, N., Carreiro, M., Castel, B., Caudrelier, L., Cougoul, P., Danu, A., Delavigne, K., Dingremont, C., Faurie, T., Gaches, F., Gaspard, M.‐H., Gaudin, C., Godel‐Labouret, A., Giraud, P., Hadj‐Khelifa, S., Hebraud, B., Khatibi, S., Leplay, L., Leveneur, Y., Limal, N., Ollier, S., Madaule, S., Marchou, B., Martel, C., Martin‐Blondel, G., Montane De La Roque, P., Michaud, M., Moeglin, J., Nuccio, F., Prudhomme, L., Pugnet, G., Recher, C., Remy, V., Sailler, L., Sire, S., Sommet, A., Tavitian, S., Thiercelin‐Legrand, M.‐F., and Vaillant, W.

Published
2018
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13. Tolérance et efficacité des thérapies ciblées prescrites pour des indications hors AMM dans les maladies auto-immunes systémiques réfractaires : données des 187 premiers patients inclus dans le registre TATA (TArgeted Therapy in Autoimmune Diseases)

Author

Gottenberg, J.E., primary, Kane, B.S., additional, Allenbach, Y., additional, Mékinian, A., additional, Amoura, Z., additional, Cacoub, P., additional, Cornec, D., additional, Hachulla, E., additional, Quartier, P., additional, Richez, C., additional, Seror, R., additional, Terrier, B., additional, Pensec, V. Devauchelle, additional, Henry, J., additional, Gatfosse, M., additional, Bouillet, L., additional, Gaigneux, E., additional, André, V., additional, Saunier, A., additional, Desmurs, M., additional, Poulet, A., additional, Bienvenu, B., additional, Truchetet, M.E., additional, Michaud, M., additional, Larroche, C., additional, Dellal, A., additional, Leurs, A., additional, Ottaviani, S., additional, Nielly, H., additional, Vial, G., additional, Jaussaud, R., additional, Rouviere, B., additional, Jeandel, C., additional, Guffroy, A., additional, Jouvray, M., additional, Gramont, B., additional, Lavigne, C., additional, Marotte, H., additional, Moulinet, T., additional, Pers, Y., additional, Queyrel-Moranne, V., additional, Maalouf, G., additional, Bolko, L., additional, Rein, C., additional, Legendre, P., additional, Meyer, A., additional, Sibilia, J., additional, Yahia, S. Ahmed, additional, Kleinmann, J.F., additional, and Mariette, X., additional

Published
2023
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16. Annular Dump Diffuser and Deswirl System for Back-Pressure Control in Engine-Scale Transonic Annular Cascade

Author

Michaud, M, Ornano, F, and Povey, T

Subjects
Mechanical Engineering
Abstract

A common requirement for turbomachinery testing facilities is the ability to independently control Mach number and Reynolds number. In practice, this means independent control of the inlet total pressure and exit static pressure in a test facility. In this paper, we present a solution to this problem with particular applicability to large-scale annular test facilities. We describe the design and commissioning of a combined annular dump diffuser and deswirl system for back-pressure control in environments with high-whirl transonic flow. The particular application was an annular cascade of nozzle guide vanes from a current civil engine. The purpose was to provide independent control of Mach number and Reynolds number, by controlling the back-pressure in the intermediate annular plenum which forms the dump diffuser. The dump diffuser is necessary to facilitate optical and probe access (without interaction effects) and to reduce the risk of exit static pressure disturbances (due to particular duct design). The system has been installed and validated in the engine component aerothermal (ECAT) facility at the University of Oxford. In this implementation of the system, the high-whirl transonic flow from the nozzle guide vanes passes through a short, parallel annular duct, and is dumped into an annular plenum, before being re-accelerated into a deswirl vane ring. The deswirl ring turns the flow to the axial direction. The flow is then discharged through a variable choke plate into a silencer. Conditioning the flow to have zero whirl at the choke plate reduces the sensitivity of the choke plate effective blockage to the whirl angle. The design, deswirl vane aerodynamic performance, and overall system performance are assessed with detailed experiments and 3D unsteady computational fluid dynamics predictions. The control of high-whirl transonic flow is notoriously challenging, and the deswirl system has application to exhaust conditioning in a number of applications including annular cascade experiments and rocket turbo-pump exhaust systems.

Published
2022
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24. Impact clinique et pronostique d’une cryoglobulinémie et d’une cryofibrinogénémie au cours de la sclérodermie systémique

Author

De Almeida Chaves, S., primary, Puissant, B., additional, Porel, T., additional, Bories, E., additional, Adoue, D., additional, Astudillo, L., additional, Alric, L., additional, Huart, A., additional, Michaud, M., additional, Ribes, D., additional, Prevot, G., additional, Sailler, L., additional, Gaches, F., additional, and Pugnet, G., additional

Published
2022
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25. Épidémiologie descriptive de l’atteinte cardiaque sévère dans la sclérodermie systémique : étude rétrospective bicentrique sur 459 patients

Author

Bories, E., primary, De Almeida, S., additional, Porel, T., additional, Alric, L., additional, Astudillo, L., additional, Gaches, F., additional, Michaud, M., additional, Catros, F., additional, Prevot, G., additional, Sailler, L., additional, Adoue, D., additional, Lairez, O., additional, and Pugnet, G., additional

Published
2022
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26. AB0651 Clinical Impact and Prognosis of cryoglobulinemia and cryofibrinogenemia in Systemic Sclerosis

Author

De Almeida Chaves, S., primary, Benedicte, P., additional, Porel, T., additional, Bories, E., additional, Adoue, D., additional, Astudillo, L., additional, Alric, L., additional, Huart, A., additional, Michaud, M., additional, Ribes, D., additional, Prevot, G., additional, Sailler, L., additional, Gaches, F., additional, and Pugnet, G., additional

Published
2022
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27. POS0711 TOLERANCE AND EFFICACY OF TARGETED THERAPIES PRESCRIBED FOR OFF-LABEL INDICATIONS IN REFRACTORY SYSTEMIC AUTOIMMUNE DISEASES: DATA OF THE FIRST 100 PATIENTS ENROLLED IN THE TATA REGISTRY (TARGETED THERAPY IN AUTOIMMUNE DISEASES)

Author

Gottenberg, J. E., primary, Chaudier, A., additional, Allenbach, Y., additional, Mekinian, A., additional, Amoura, Z., additional, Cacoub, P., additional, Cornec, D., additional, Hachulla, E., additional, Quartier, P., additional, Melki, I., additional, Richez, C., additional, Seror, R., additional, Terrier, B., additional, Devauchelle-Pensec, V., additional, Henry, J., additional, Gatfosse, M., additional, Bouillet, L., additional, Gaigneux, E., additional, Andre, V., additional, Baulier, G., additional, Saunier, A., additional, Desmurs, M., additional, Poulet, A., additional, Ete, M., additional, Truchetet, M. E., additional, Michaud, M., additional, Larroche, C., additional, Dellal, A., additional, Leurs, A., additional, Ottaviani, S., additional, Nielly, H., additional, Vial, G., additional, Jaussaud, R., additional, Rouviere, B., additional, Jeandel, P. Y., additional, Guffroy, A., additional, Korganow, A. S., additional, Jouvray, M., additional, Meyer, A., additional, Chatelus, E., additional, Sordet, C., additional, Felten, R., additional, Sibilia, J., additional, Ahmed Yahia, S., additional, Kleinmann, J. F., additional, and Mariette, X., additional

Published
2022
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28. POS0884 DESCRIPTIVE EPIDEMIOLOGY OF SEVERE CARDIAC INVOLVEMENT IN SYSTEMIC SCLEROSIS: A BICENTRIC RETROSPECTIVE STUDY ON 459 PATIENTS

Author

Bories, E., primary, DE Almeida Chaves, S., additional, Porel, T., additional, Alric, L., additional, Astudillo, L., additional, Gaches, F., additional, Michaud, M., additional, Catros, F., additional, Prevot, G., additional, Sailler, L., additional, Adoue, D., additional, Lairez, O., additional, and Pugnet, G., additional

Published
2022
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30. Automatic mapping and innovative on-demand mapping services at IGN France

Author

Lafay, S., Braun, A., Chandler, D., Michaud, M., Ricaud, L., and Mustiere, S.

Subjects
Digital map services -- Analysis, Geography
Abstract

Mapmaking has become widespread through the Internet, resulting in a wide range of cartographic quality. To achieve better quality, mapmaking needs tools and online services for intuitive and efficient on-demand mapping. A project team at IGN, the French National Mapping Agency, is working on producing a digital cartographic model (DCM) from various existing databases and maps on which such tools and services are based. This DCM ranges from detailed topographic maps to small general road maps. GeoServer Web Map Service capabilities were used extensively to produce quality maps with various legends. Special care was taken to make a default legend suitable for customer data overlays, both on-screen and on paper. Web-based interface prototypes were built to guide users in choosing colors and creating their own original map legends. Users can also rely on a growing catalog of harmonious color palettes and map samples as sources of inspiration. Keywords: automatic mapping; geographic database; on-demand mapping; WMS; SLD, 1. Introduction Modern cartography has been evolving quickly thanks to new tools and devices. Mapmaking has become widespread through the Internet. The growing possibility of creating maps on the Internet [...]

Published
2015
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36. Tolérance et efficacité des thérapies ciblées prescrites hors AMM au cours des maladies auto-immunes systémiques réfractaires : données des 100 premiers patients inclus dans le registre TATA (TArgeted Therapy in Autoimmune Diseases)

Author

Gottenberg, J.E., primary, Chaudier, A., additional, Allenbach, Y., additional, Mékinian, A., additional, Richez, C., additional, Truchetet, M.E., additional, Hachulla, E., additional, Amoura, Z., additional, Devauchelle Pensec, V., additional, Cornec, D., additional, Quartier, P., additional, Terrier, B., additional, Henry, J., additional, Gatfosse, M., additional, Jouvray, M., additional, Bouillet, L., additional, Gaigneux, E., additional, André, V., additional, Baulier, G., additional, Saunier, A., additional, Desmurs, M., additional, Poulet, A., additional, Ete, M., additional, Bienvenu, B., additional, Michaud, M., additional, Larroche, C., additional, Dellal, A., additional, Leurs, A., additional, Ottaviani, S., additional, Nielly, H., additional, Vial, G., additional, Jaussaud, R., additional, Moulinet, T., additional, Guffroy, A., additional, Meyer, A., additional, Chatelus, E., additional, Sordet, C., additional, Felten, R., additional, Ahmed Yahia, S., additional, Kleinmann, J.F., additional, Cacoub, P., additional, Seror, R., additional, and Mariette, X., additional

Published
2021
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48. Long-term benefit of enzyme replacement therapy with alglucosidase alfa in adults with Pompe disease: prospective analysis from the French Pompe Registry

Author

Semplicini, C., de Antonio, M., Taouagh, N., Béhin, A., Bouhour, F., Echaniz-Laguna, A., Magot, A., Nadaj-Pakleza, A., Orlikowski, D., Sacconi, S., Salort-Campana, E., Solé, G., Tard, Celine, Zagnoli, F., Jean-Yves, H., Hamroun, D., Laforêt, P., Attarian, S., Aubé-Nathier, A. C., Arrassi, A., Bassez, G., Bedat-Millet, A. L., Bouibede, F., Boyer, F. C., Caillaud, C., Canal, A., Carlier, R. Y., Chanson, J. B., Chapon, F., Cintas, P., Deibener-Kaminsky, J., Demurger, F., Desnuelle, C., Durieu, I., Eymard, B., Feasson, L., Fournier, M., Froissart, R., Furby, A., Garcia, P. Y., Germain, D. P., Ghorab, K., Morales, R. J., Krim, E., Labauge, P., Lacour, A., Lagrange, E., Lefeuvre, C., Leguy-Seguin, V., Leonard-Louis, S., Magy, L., Masseau, A., Michaud, M., Minot-Myhié, M. C., Nicolas, G., Nollet, S., Not, A., Noury, J. B., Ollivier, G., Péréon, Y., Perez, Thierry, Perniconi, B., Piraud, M., Petiot, P., Pouget, J., Praline, J., Prigent, H., Renard, D., Spinazzi, M., Stojkovic, T., Taithe, F., Tiffreau, Vincent, Vincent, D., Lille Neurosciences & Cognition - U 1172 (LilNCog), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Unité de Recherche Pluridisciplinaire Sport, Santé, Société (URePSSS) - ULR 7369 - ULR 4488 (URePSSS), and Université d'Artois (UA)-Université du Littoral Côte d'Opale (ULCO)-Université de Lille

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Registry, alglucosidase alfa, Adolescent, [SDV]Life Sciences [q-bio], Walk Test, Disease, Sitting, 03 medical and health sciences, FEV1/FVC ratio, Young Adult, Glycogen storage disease type II, Genetics, medicine, Humans, Respiratory function, Prospective Studies, Registries, Child, Alglucosidase alfa, Genetics (clinical), 030304 developmental biology, Aged, 0303 health sciences, late onset Pompe disease, business.industry, 030305 genetics & heredity, alpha-Glucosidases, Enzyme replacement therapy, Middle Aged, medicine.disease, Respiratory Function Tests, Treatment Outcome, Ceiling effect, Female, France, business, medicine.drug, enzyme replacement therapy
Abstract

Despite a wide clinical spectrum, the adult form of Pompe disease is the most common one, and represents more than 90% of diagnosed patients in France. Since the marketing of enzyme replacement therapy (alglucosidase alfa, Myozyme), all reports to date in adults demonstrated an improvement of the walking distance, and a trend toward stabilization of respiratory function, but the majority of these studies were less than 5 years of duration. We report here the findings from 158 treated patients included in the French Pompe Registry, who underwent regular clinical assessments based on commonly used standardized tests (6-minute walking test, MFM scale, sitting vital capacity, MIP and MEP). For longitudinal analyses, the linear mixed effects models were used to assess trends in primary endpoints over time under ERT. A two-phase model better described the changes in distance traveled in the 6-minute walk test and MFM. 6MWT showed an initial significant increase (1.4% ± 0.5/year) followed by a progressive decline (-2.3%/year), with a cut-off point at 2.2 years. A similar pattern was observed in total MFM score (6.6% ± 2.3/year followed by a - 1.1%/year decline after 0.5 years). A single-phase decline with a slope of -0.9 ± 0.1%/year (P < .001) was observed for FVC, and MEP remained stable over the all duration of follow-up. This study provides further evidence that ERT improves walking abilities and likely stabilizes respiratory function in adult patients with Pompe disease, with a ceiling effect for the 6MWT in the first 3 years of treatment.

Published
2020
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49. Unexplained Splenomegalies : Interim Data of the French Prospective Multi-Center Splenomegaly Study (SMS)

Author

Denis, G., Terriou, L., Séné, T., Costello, R., Urbanski, G., Michaud, M., Sanhes, L., Bauduer, F., Lagadec, A., Rose, C., Berger, M.G., Role of intra-Clonal Heterogeneity and Leukemic environment in ThErapy Resistance of chronic leukemias (CHELTER), Université Clermont Auvergne [2017-2020] (UCA [2017-2020]), and CHADEYRON, DOMINIQUE

Subjects
[SDV.MHEP.HEM] Life Sciences [q-bio]/Human health and pathology/Hematology, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2020

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