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    Le design au service du territoire : retour sur la recherche in situ menée par le DSAAMaster « Produire autrement » de l’ENSAAMA à Granville.

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    ensaama.netCet article est composé à partir des articles et retours d’expériences des étudiants et de la table-ronde du 02 avril2025, à l’ENSAAMA, animée par Hélène Monnet-Cantagrel (enseignante en Humanités) et accueillant Sandrine Yvenou,fondatrice de l’établissement Dame Gourmande qui a accueilli un des projets, les étudiants Margaux Routaboul, SiméonMalaval, et Samuel Galmiche, leur professeur Richard Devinast, enseignant de Design Produit et référent duDSAA/Master et Marine Catel, designer, chercheuse et porteuse du projet Accélérateur de Transfo fédérant ce partenariat

    Recurrent penile and oral aphthosis revealing an LGL T-cell lymphoproliferative disorder

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    International audienceLarge granular T-cell lymphoproliferative disorders are rare and often silent, making diagnosis difficult. We report a 26-year-old man with recurrent oral and genital aphthous ulcers initially diagnosed as idiopathic bipolar aphthosis. Colchicine therapy induced neutropenia, prompting investigations that revealed a clonal large granular T-cell lymphoproliferative disorder. This case underscores the importance of considering hematological disease when recurrent mucosal aphthosis is associated with unexplained neutropenia

    Mind the Gap: What Missing Pressures Tell Us about BEST-CLI

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    International audienc

    Les paradoxes du droit à l’avortement en Amérique latine : entre reconnaissance, contrôle et stigmatisation

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    International audienceAu cours des dernières années, l’Amérique latine s’est imposée comme un des laboratoirespolitiques les plus dynamiques en matière de droits sexuels et reproductifs. En 2020, lesmobilisations massives de femmes argentines revendiquaient dans les rues de Buenos Airesla pleine reconnaissance du droit à l’avortement par le gouvernement d’Alberto Fernández.Deux ans plus tard, la Cour constitutionnelle colombienne a opéré un revirement historiqueen dépénalisant l’interruption volontaire de grossesse (IVG) jusqu’à la vingt-quatrième semainede gestation, seuil de la viabilité foetale hors de l’utérus établi selon des critères médicaux.A titre de comparaison, la législation française fixe cette limite à quatorze semaines degestation, ce qui souligne la portée inédite de la décision prise par la justice colombienne.A l’inverse, le processus constitutionnel chilien a révélé la persistance des résistancesconservatrices au sein de l’Assemblée constituante. En 2023, les électeurs ont rejeté lanouvelle proposition de Constitution, notamment en raison de la volonté de ses rédacteursde restreindre à nouveau l’accès à l’avortement

    Clinical Manifestations of Emerging Trichosporon spp. Infections, France

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    International audienceFungi in the family Trichosporonaceae are rarely involved in invasive disease but are frequently associated with colonization or respiratory allergic infection. Trichosporonaceae exhibit intrinsic resistance to echinocandin antimicrobial drugs, posing challenges for treatment and contributing to high mortality rates. We complied a nationwide analysis of 112 cases of invasive disease caused by Trichosporon spp. and related fungi, diagnosed in France over 20 years, that combined clinical data, susceptibility profiles, and molecular identification. We identified 12 species; T. asahii was the most common species recovered, and the new species T. austroamericanum was next. Comparison of clinical data highlighted species and genotypic differences, such as a much higher proportion of children infected by T. asahii and major differences in antimicrobial drug susceptibility. Correct identification is not only of epidemiologic interest but also necessary for patient management because of the varying clinical and microbiological characteristics found in different species

    Prognostic impact of late gadolinium enhancement granularity using cardiovascular magnetic resonance in end-stage hypertrophic cardiomyopathy

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    International audienceBackground: End-stage hypertrophic cardiomyopathy (HCM) is a distinct and advanced form of HCM, defined by a left ventricular ejection fraction (LVEF) < 50% and associated with a markedly poor prognosis. Evidence on the prognostic relevance of LGE and its key features in end-stage HCM remains limited. We aim to evaluate the prognostic value of the LGE granularity model including its location, extent and pattern in end-stage HCM patients.Methods: All patients referred for cardiovascular magnetic resonance (CMR) assessment of HCM at three French university hospitals between 2008 and 2024 were retrospectively screened. All patients with HCM and LVEF < 50% were included. The “LGE granularity model” was defined as a model combining LGE extent (focal vs. multifocal involvement), location (septal vs. other), and pattern (subepicardial vs. midwall). The primary endpoint was all-cause mortality using the National Registry of Death.Results: Among 2,873 patients with HCM, 691 (24%) with end-stage HCM were included (53±7 years, 54% male). At the time of CMR, 30% of patients presented with heart failure symptoms classified as NYHA > II and 26% had a history of atrial fibrillation. After a median follow-up of 9 years (IQR 6–11), 226 patients died (32%). LGE was observed in 259 patients (37%) and was strongly associated with mortality (HR: 3.37, [95% CI: 2.57-4.42], p< 0.001), even after adjustment for traditional prognostic factors (HR: 1.52, [95% CI: 1.07-2.18], p=0.02). In sub-group analysis, LGE presence was associated with all-cause mortality in patients with LVEF >40% (HR: 3.32 [95% CI: 1.61-6.45], p< 0.001) but was not in patients with LVEF < 40% (HR: 0.98 [95% CI: 0.73-1.32], p=0.90). Each LGE granularity component was independently associated with mortality after adjustment with traditional prognostic factors: LGE extent (HR: 2.85, [95% CI: 1.03-7.85], p=0.02), septal location (HR: 1.73, [95% CI: 1.10-2.73], p< 0.001), and midwall pattern (HR: 4.15, [95% CI: 1.79-9.61], p< 0.001). The LGE granularity model improved risk reclassification and discrimination compared to traditional prognostic factors (NRI: 32%; IDI: 10%, both p< 0.001; global Chi-square: 139 to 167, p< 0.001).Conclusion: In this large multicenter cohort of end-stage HCM patients, the LGE granularity model integrating the LGE extent, location, and pattern provided strong and independent prognostic value beyond traditional prognostic factors

    Changes in phenology mediate vertebrate population responses to temperature globally

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    International audiencePhenotypic responses to climate affect individual fitness, but the extent to which this translates into effects on population dynamics remains poorly understood. We assemble 213 time series on phenotypes and population sizes of wild vertebrates globally and match them with local climate data. Our meta-analysis shows that morphological traits are mostly climate insensitive. However, phenology is earlier in warmer-than-average years, which contributes positively to population growth in most species. At lower latitudes, temperature has weaker effects on phenology but stronger direct negative effects on population growth, likely because these populations are less capable of tracking climate via plasticity. Variation in the phenology-mediated effect of temperature on population growth cannot be explained by latitude, generation time, migratory mode, or diet. This suggests that simple relationships between species characteristics and population responses to warming may not occur in nature. Instead, we may need to embrace ecological complexity by considering local-scale predictors that capture intra-specific variation

    Adult male patients with DKC1 mutations present early-onset pulmonary fibrosis and severe prognosis

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    International audienceBackground Mutations in telomere-related genes (TRGs) are the main cause of monogenic familial pulmonary fibrosis. Dyskerin, encoded by the X-localised gene DKC1 , is involved in telomere maintenance. Methods This retrospective study aimed to further characterise the pulmonary phenotype of DKC1 -deficient patients with pulmonary fibrosis identified between 2010 and 2025 in our laboratory. Results We reported eight, as yet undescribed, to our knowledge, probands affected by pulmonary fibrosis associated with X-linked DKC1 deficiency. The median age at interstitial lung disease (ILD) diagnosis was 47 years. Four had idiopathic pulmonary fibrosis, two unclassifiable fibrosis, one idiopathic nonspecific interstitial pneumonia and one unknown. All patients displayed ectodermal abnormalities (premature hair greying, nail dystrophies, reticulated hyper- or hypopigmentation, tooth abnormalities and oral leukoplasia). Haematological abnormalities were found in four patients. Seven patients died during follow-up and one patient received a transplant. Median survival after ILD diagnosis was 22 months (range 3–81 months). Conclusions Compared with patients with pulmonary fibrosis associated with other TRG variants, those with pulmonary fibrosis associated with X-linked recessive DKC1 variants are younger and have a shorter survival time. Early identification and referral of these patients to an expert transplantation centre should be considered

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