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295 results on '"Labarthe, F"'

4. A Multiplatform Metabolomics Approach to Characterize Plasma Levels of Phenylalanine and Tyrosine in Phenylketonuria

Author

Blasco, H., Veyrat-Durebex, C., Bertrand, M., Patin, F., Labarthe, F., Henique, H., Emond, P., Andres, C. R., Antar, C., Landon, C., Nadal-Desbarats, L., Maillot, F., Baumgartner, Matthias R., Series editor, Patterson, Marc, Series editor, Rahman, Shamima, Series editor, Peters, Verena, Series editor, Morava, Eva, Editor-in-chief, Zschocke, Johannes, Series editor, and Baumgartner, Matthias, editor

Published
2017
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21. Transition in health care from childhood to adulthood (TCA) for lysosomal diseases patients in France. Current state and priorities : The TENALYS study

Author

Belmatoug, N., Marcel, C., Arnoux, A., Brassier, A., Pichard, S., Feillet, F., Labarthe, F., Chabrol, B., Berger, M., Héron, B., Genevaz, D., CHADEYRON, DOMINIQUE, Role of intra-Clonal Heterogeneity and Leukemic environment in ThErapy Resistance of chronic leukemias (CHELTER), and Université Clermont Auvergne [2017-2020] (UCA [2017-2020])

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

24. Les déclinaisons de l’empowerment en régime numérique

Author

Labarthe, F., Trainoir, Marianne, Centre de Recherche sur l'Education, les apprentissages et la didactique (CREAD EA 3875), Université de Brest (UBO)-Université de Rennes 2 (UR2), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut Brestois des Sciences de l'Homme et de la Société (IBSHS), and Université de Brest (UBO)

Subjects
[SHS.EDU]Humanities and Social Sciences/Education, ComputingMilieux_MISCELLANEOUS
Abstract

National audience

Published
2019

28. HIGHLY RESPONSIVE AND ACCURATE TEMPERATURE MEASUREMENTS IN ORTHOGONAL CUTTING THROUGH INNOVATIVE SINGLE LEG THERMOCOUPLE.

Author

Hamm, I., Rossi, F., Poulachon, G., Marcon, B., Birembaux, H., Maury, N., Valadon, S., and Labarthe, F.

Subjects
TEMPERATURE measurements, MECHANICAL energy, TITANIUM alloys, THERMOCOUPLES, ENERGY conversion, WIRE, THERMAL diffusivity
Abstract

During machining, most of the thermal sources are resulting from the conversion of mechanical energy during the chip shearing mechanism and the intense frictions between the tool, the chip, and the machined surface. Thermal gradients are high and localized, especially for low thermal diffusivity material like titanium alloys. Moreover, heat rates come close to few million degrees per second in the shearing zones. Even though several authors performed thermography techniques to determine the temperature distribution into the machined material, their results remain underestimated due to experimental limitations. In this research, a new temperature measurement technique has been developed based on a single wire thermocouple to measure at one exact location both the temperature and the heating rate during orthogonal cutting test. The tests are performed with uncut chip thicknesses of 0.020 mm and 0.100 mm and with a cutting speed of 120 m/min with two different cutting angles. Results are discussed and compared to prior studies. [ABSTRACT FROM AUTHOR]

Published
2021
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32. Management of phenylketonuria in Europe: Survey results from 19 countries

Author

Ozturk, YEŞİM, Niinikoski, H., Bonnemains, C., Marioli, S., Barat, P., De Parscau, L., Meyer, M., Bedu, A., Güttler, F., Pazdirkova, R., Prochazkova, D., Sarnavka, V., Baric, I., Toromanovic, A., Tahirovic, H., Scholl-Bürgi, S., Karall, D., Van Spronsen, F.J., Trefz, Friedrich K., Giovannini, Marcello, Feillet, François, Demirkol, M., Bélanger-Quintana, A., Blau, Nenad, Aydin, H., Coskun, T., Dursun, A., Kalkanoglu, S.H.S., Tokatli, A., Eminoglu, F.T., Hasanoglu, A., Baumgartner, M., Onenli-Mungan, N., Yüksel, B., Gil-Ortega, D., Odent, S., Eyer, D., Labarthe, F., Hennermann, J.B., Mönch, E., Stolz, S., Spiekerkötter, U., Knerr, I., Schwab, K.O., Kreuder, J., Ullrich, K., Das, A.M., Burgard, P., Kon-Stantopoulou, V., Lindner, M., Müller, E., Haase, C., Beblo, S., Weigel, J., Plötzch, S., Muntau, A., Weglage, J., Marquardt, J., Scheible, D., Clemens, P., Schulpis, K.H., Papadia, F., Salardi, S., Meli, C., Donati, M.A., Procopio, E., Cerone, R., Riva, E., Giovannini, M., Paci, S., Carbone, M.T., Burlina, A., Lapichino, L., Cotugno, G., Leuzzi, V., Rubio-Gozalbo, E., De Vries, M., De Klerk, J.B.C., Walter, J., Cleary, M.A., Schwann, B., Robinson, P., Galloway, P., Hendriksz, C.J., Iversen, K., Wiig, I., Jørgensen, J., Milanowski, A., Nowacka, M., Djordjevic, M., Laketa, C., Gutiérrez-Junquera, C., Márquez-Armenteros, A., Vilaseca Busca, M.A., Campistol Plana, J., Peña-Quintana, L., Valverde, F.S., Gonzalez-Lamuno, D., Couce-Pico, M.L., Dalmau Serra, J., Baldellou-Vazquez, A., Garcia-Jimenez, M.C., Papadopoulou, D., Almm, J., Okur, I., Süheyl, E.F., Tumer, L., Aydogdu, S., Aktuglu-Zeybek, A.C., Cansever, S., Arslan, N., Erdur, B., Coker, M., Kalkan, U.S., Hizel-Bülbül, S., Tanzer, F., MacDonald, Anita, MacDonald, A., Chakrapani, A., Gomez, A.R., Fouilhoux, A., Chabrol, B., Wagner, K., Billette De Villemeur, T., De Lonlay-Debeney, P., Ogier De Baulny, H., Halldin Stenlid, M., Nuoffer, J.M., Rohrbach, M., Faculteit Medische Wetenschappen/UMCG, Center for Liver, Digestive and Metabolic Diseases (CLDM), Kindergeneeskunde, RS: GROW - School for Oncology and Reproduction, University of Zurich, and Blau, N

Subjects
Dieticians, Pediatrics, 1303 Biochemistry, phenylalanine, Endocrinology, Diabetes and Metabolism, Prevalence, CHILDREN, Biochemistry, RECOMMENDATIONS, Endocrinology, Hyperphenylalaninemia, DIETARY CONTROL, Phenylketonurias, Surveys and Questionnaires, Epidemiology, Registries, guidelines, BH4, 1310 Endocrinology, Europe, 2712 Endocrinology, Diabetes and Metabolism, Child, Preschool, 10076 Center for Integrative Human Physiology, CONCURRENT PHENYLALANINE LEVELS, PKU, Adult, congenital, hereditary, and neonatal diseases and abnormalities, medicine.medical_specialty, Health Planning Guidelines, NEUTRAL AMINO-ACIDS, MEDLINE, 610 Medicine & health, Survey result, 1311 Genetics, Age groups, 1312 Molecular Biology, Genetics, medicine, Humans, Molecular Biology, hyperphenylalaninemia, business.industry, Infant, Newborn, nutritional and metabolic diseases, bh4, diet, pku, Guideline, medicine.disease, TRANSPORT, phenylketonuria, European countries, tetrahydrobiopterin, 10036 Medical Clinic, Health Care Surveys, 570 Life sciences, biology, business, Follow-Up Studies
Abstract

To gain better insight in the most current diagnosis and treatment practices for phenylketonuria (PKU) from a broad group of experts, a European PKU survey was performed. The questionnaire, consisting of 33 questions, was sent to 243 PKU professionals in 165 PKU centers in 23 European countries. The responses were compiled and descriptive analyses were performed. One hundred and one questionnaires were returned by 93/165 centers (56%) from 19/23 European countries (83%). The majority of respondents (77%) managed patients of all age groups and more than 90% of PKU teams included physicians or dieticians/nutritionists. The greatest variability existed especially in the definition of PKU phenotypes, therapeutic blood phenylalanine (Phe) target concentrations, and follow-up practices for PKU patients. The tetrahydrobiopterin (BH4 ; sapropterin) loading test was performed by 54% of respondents, of which 61% applied a single dose test (20mg/kg over 24h). BH4 was reported as a treatment option by 34%. This survey documents differences in diagnostic and treatment practices for PKU patients in European centers. In particular, recommendations for the treatment decision varied greatly between different European countries. There is an urgent need to pool long-term data in PKU registries in order to generate an evidence-based international guideline. Copyright (c) 2009 Elsevier Inc. All rights reserved.

Published
2010

34. Effects of miglustat therapy on neurological disorder and survival in early-infantile Niemann-Pick disease type C: A national French retrospective study

Author

Freihuber, C., primary, Dahmani, B., additional, Brassier, A., additional, Broue, P., additional, Cances, C., additional, Chabrol, B., additional, Eyer, D., additional, Labarthe, F., additional, Latour, P., additional, Levade, T., additional, Pichard, S., additional, Sevin, C., additional, Vanier, M.T., additional, and Héron, B., additional

Published
2017
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37. Lorsque l’enfant parait … ou l’histoire d’une infection congénitale solognote improbable

Author

Desoubeaux, Guillaume, Maakaroun-Vermesse, Z, Lanotte, Philippe, Chaussade, Hélène, Perez Simarro, P., Laulier, D., Bailly, E., Suc, A.C., Guennoc, Anne-Marie, De Toffol, Bertrand, Goudeau, Alain, Bernard, L., Paris, Luc, Labarthe, F., Chandenier, J, Infectiologie et Santé Publique (UMR ISP), Institut National de la Recherche Agronomique (INRA)-Université de Tours, Société Française de Parasitologie (SFP). FRA., ProdInra, Migration, and Institut National de la Recherche Agronomique (INRA)-Université de Tours (UT)

Subjects
[SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, [SDV.MP]Life Sciences [q-bio]/Microbiology and Parasitology, [SDV.MP] Life Sciences [q-bio]/Microbiology and Parasitology, ComputingMilieux_MISCELLANEOUS, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

National audience

Published
2014

39. Utilisation d’une approche métabolomique multimodale pour caractériser les concentrations plasmatiques de phénylalanine et de tyrosine dans la phénylcétonurie

Author

Blasco, H., primary, Veyrat-Durebex, C., additional, Bertrand, M., additional, Patin, F., additional, Labarthe, F., additional, Henique, H., additional, Emond, P., additional, Andres, C.R., additional, Antar, C., additional, Landon, C., additional, Nadal-Desbarats, L., additional, and Maillot, F., additional

Published
2016
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41. La maladie de Wilson : une urgence diagnostique

Author

Bauvois, A., primary, Bayer, G., additional, Labarthe, F., additional, Maillot, F., additional, Mankikian, J., additional, Poujois, A., additional, Tardieu, M., additional, Woimant, F., additional, and Legras, A., additional

Published
2015
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42. Mutation spectrum in the French cohort of galactosemic patients and structural simulation of 27 novel missense variations

Author

Boutron, A, Marabotti, Anna, Facchiano, A, Cheillan, D, Zater, M, Oliveira, C, Costa, C, Labrune, P, Brivet, M, Loisel, N, Morin, G, Barth, M, Altuzarra, C, Mesli, S, Rigalleau, V, Rhead, Mh, de Schrevel, G, Thauvin Robinet, C, Santos, A, Dobbelaere, D, Mention, K, Guffon, N, Cano, A, Feillet, F, Krempf, P, Pitelet, G, Bernard, O, Hermeziu, B, Gonzalez, E, Habes, D, Jacquemin, E, de Baulny, H, Roche, S, Debray, D, de Lonlay, P, Servais, A, Touati, G, Bednarek, N, Garnotel, R, Devaux, Am, Gay, C, Eyer, D, Baruteau, J, Broué, P, Labarthe, F, and Maillot, F.

Subjects
Adult, Galactosemias, Male, Adolescent, Endocrinology, Diabetes and Metabolism, In silico, DNA Mutational Analysis, Biology, medicine.disease_cause, Biochemistry, Cohort Studies, Exon, Endocrinology, Genetics, medicine, Humans, UTP-Hexose-1-Phosphate Uridylyltransferase, Missense mutation, Homology modeling, Child, Molecular Biology, Mutation, Protein Stability, Galactosemia, Infant, Exons, Middle Aged, medicine.disease, Introns, Molecular Docking Simulation, Protein Subunits, Phenotype, Child, Preschool, Female, Allelic heterogeneity, France
Abstract

Background Classic galactosemia refers to galactose-1-phosphate uridyltransferase (GALT) deficiency and is characterized by long-term complications of unknown mechanism and high allelic heterogeneity of GALT gene. Aim To report molecular characterization of GALT variations in 210 French families, to analyze the structural effects of novel missense variations and to assess informativity of structural data in predicting outcome. Methods Sequencing of exons and intron–exon junctions of GALT gene was completed in unsolved cases by analysis of a long range PCR product. Structural consequences of novel missense variations were predicted using a homology model of GALT protein and a semi-automated analysis which integrates simulation of variations, structural analyses and two web servers dedicated to identify mutation-induced change of protein stability. Results Forty four novel variations were identified, among them 27 nucleotide substitutions. In silico modeling of these missense variations showed that 12 variations are predicted to impair subunit interactions and/or active site conformation and that 23 variations modify H-bond or salt-bridge networks. Twenty variations decrease the global stability of the protein. Five variations had apparently no structural effect. Conclusion Our results expand the mutation spectrum in GALT gene and the list of GALT variations analyzed at the structural level, providing new data to assess the pathophysiology of galactosemia.

Published
2012

48. SFP CO-09 - L’allaitement maternel dans la phénylcétonurie

Author

Livrozet, C., primary, Tardieu, M., additional, Odent, S., additional, Barth, M., additional, De Parscau, L., additional, Kuster, A., additional, Hankard, R., additional, Saint Romas, E., additional, Bigot, S., additional, Jouault, C., additional, Leven, M., additional, Boscher, V., additional, Hermouet, P., additional, and Labarthe, F., additional

Published
2014
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