22 research outputs found

    TRADUCTION DU CODE DE COMMERCE FRANÇAIS EN ESPAGNOL VERSION BILINGUE

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    Traduction bilingue en espagnol du code de commerce français en vigueur le 1er juillet 2013, prĂ©sentĂ©e en bi-texteDepuis 1999, le Gouvernement français fait procĂ©der Ă  la traduction des grands codes français en anglais et en espagnol aux fins de publication sur le site http://www.legifrance.gouv.fr. Pour assurer leur qualitĂ©, ces traductions font l'objet d'un accompagnement scientifique associant des experts, juristes bilingues, spĂ©cialistes de la discipline qui fait l'objet du texte traduit, et une ingĂ©nierie spĂ©cialisĂ©e, une juriste-linguiste, spĂ©cialiste de traduction et de terminologie juridique. La juriste-linguiste a pour mission de faciliter la collaboration et les Ă©changes entre les linguistes et les juristes, dont les approches mĂ©thodologiques et conceptuelles diffĂšrent. Sa connaissance des deux domaines scientifiques facilite le rapprochement des points de vue et le travail transdisciplinaire. La traduction est prĂ©cĂ©dĂ©e d'opĂ©rations de prĂ©-traduction menĂ©es par l'ingĂ©nieur qui conduisent notamment Ă  Ă©laborer un lexique d'Ă©quivalences terminologiques validĂ© conjointement par les experts et les traducteurs de la combinaison linguistique retenue. Cet outil est indispensable Ă  la traduction d'un texte long intĂ©grĂ© dans un programme comportant plus de 10 codes, qui fait par ailleurs l'objet de mises Ă  jour. La traduction rĂ©alisĂ©e est soumise Ă  l'expertise des juristes et au contrĂŽle qualitĂ© de l'ingĂ©nieur spĂ©cialisĂ©. AprĂšs discussion et Ă©changes scientifiques, la version dĂ©finitive de la traduction est adoptĂ©e, la mĂ©moire de traduction nettoyĂ©e, ainsi que le lexique des Ă©quivalences terminologiques. En 2013-2014, les traductions du code civil et du code de commerce français en anglais et en espagnol ont Ă©tĂ© mises Ă  jour. Pour favoriser l’analyse et le dĂ©bat scientifique autour de ces traductions, et conformĂ©ment Ă  la politique de diffusion en accĂšs ouvert des donnĂ©es de la recherche produites sur fonds publics, la version bilingue de la traduction du code de commerce en espagnol est ici mise Ă  disposition de la communautĂ© scientifique, accompagnĂ©e du lexique d'Ă©quivalences terminologiques

    Relevance of C5b9 immunostaining in the diagnosis of neonatal hemochromatosis

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    BACKGROUND: Neonatal hemochromatosis caused by a gestational alloimmune mechanism or gestational alloimmune liver disease (GALD) is a rare perinatal disorder characterized by intra- and extrahepatic iron overload. It is believed to result from complement-mediated liver injury, in which the classical complement pathway is activated by maternal antibody/fetal antigen complexes, leading to hepatocyte lysis by the membrane attack complex C5b9. According to some authors, C5b9 expression in more than 75% of liver parenchyma is specific for GALD. // METHODS: We conducted a retrospective multicentric immunohistochemical study with anti-C5b9 in GALD cases (n = 25) and non-GALD cases with iron overload (n = 36) and without iron overload (n = 18). // RESULTS: C5b9 was expressed in 100% of GALD cases but involved more than 75% of the liver parenchyma in only 26% of the cases. C5b9 was detected in 26.75% of the non-GALD cases with more than 75% of positive parenchyma in maternal erythrocytic alloimmunization, herpes and enterovirus hepatitis, bile acid synthetic defect, DGUOK mutation, Gaucher disease, cystic fibrosis, and giant-cell hepatitis with autoimmune hemolytic anemia. // CONCLUSION: Diagnosis and therapeutic management of GALD cannot only be based on C5b9 expression in liver samples as it is not specific of this disease

    Erythropoietin modulation of podocalyxin and a proposed erythroblast niche

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    Epo's erythropoietic capacity is ascribed largely to its antiapoptotic actions. In part via gene profiling of bone marrow erythroblasts, Epo is now shown to selectively down-modulate the adhesion/migration factors chemokine receptor-4 (Cxcr4) and integrin alpha-4 (Itga4) and to up-modulate growth differentiation factor-3 (Gdf3), oncostatin-M (OncoM), and podocalyxin like-1 (PODXL). For PODXL, Epo dose–dependent expression of this CD34-related sialomucin was discovered in Kit+CD71high proerythroblasts and was sustained at subsequent Kit−CD71high and Ter119+ stages. In vivo, Epo markedly induced PODXL expression in these progenitors and in marrow-resident reticulocytes. This was further associated with a rapid release of PODXL+ reticulocytes to blood. As studied in erythroblasts expressing minimal Epo receptor (EpoR) alleles, efficient PODXL induction proved dependence on an EpoR-PY343 Stat5 binding site. Moreover, in mice expressing an EpoR-HM F343 allele, compromised Epo-induced PODXL expression correlated with abnormal anucleated red cell representation in marrow. By modulating this select set of cell-surface adhesion molecules and chemokines, Epo is proposed to mobilize erythroblasts from a hypothesized stromal niche and possibly promote reticulocyte egress to blood

    The CHD4-related syndrome: a comprehensive investigation of the clinical spectrum, genotype-phenotype correlations, and molecular basis

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    Sifrim-Hitz-Weiss syndrome (SIHIWES) is a recently described multisystemic neurodevelopmental disorder caused by de novo variants in CHD4. In this study, we investigated the clinical spectrum of the disorder, genotype-phenotype correlations, and the effect of different missense variants on CHD4 function
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