19 results on '"Baudet, A."'
Search Results
2. Plastic deformation effect on contact behaviour in granular materials.
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Altuhafi, F. N., Baudet, B. A., and Coop, M. R.
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Contact tests on machined and natural granite showed that extensive plastic deformation which extends to the core shape is happening before the cross-over from the behaviour of an elastic rough surface to the Hertzian behaviour of an elastic smooth contact when all asperities have yielded in the surface. The plastic deformation, which was found to take place when the estimated maximum stresses at the contact reaches about 0.6 of the material hardness, affects the behaviour during normal loading as the material will start to deform at constant stiffness after reaching these stresses. The plastic deformation during lateral loading also affects the applicability of lateral loading models. The data yielded a much lower lateral stiffness which is around one order of magnitude less than that predicted by the available contact models. [ABSTRACT FROM AUTHOR]
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- 2024
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3. Synthesis of sydnonimines from sydnones and their use for bioorthogonal release of isocyanates in cells.
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Baudet, Judith, Lesur, Emilie, Ribéraud, Maxime, Chevalier, Arnaud, D'Anfray, Timothée, Thuéry, Pierre, Audisio, Davide, and Taran, Frédéric
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SYDNONES , *ISOCYANATES , *ALIPHATIC amines , *DRUG utilization - Abstract
In this article, we report the synthesis of sydnonimines from sydnones and their use as dipoles for fast click-and-release reactions. The process relies on nucleophilic aromatic substitution of aliphatic and aromatic amines with triflated sydnones. This new methodology allowed the preparation of functionalised sydnonimine probes that are otherwise difficult to prepare. These probes were then used to release a drug and a fluorescent aromatic isocyanate inside living cells. [ABSTRACT FROM AUTHOR]
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- 2024
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4. Evolution of nano-pores in illite-dominant clay during consolidation.
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Zheng, Yanhao and Baudet, Béatrice A.
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SMALL-angle scattering , *PROGRESSIVE collapse , *CLAY , *GAS absorption & adsorption , *EVIDENCE gaps - Abstract
In this paper, the evolution of nanoscale pores, covering inter-particle pores and inter-layer pores, in illite-dominant clay during consolidation is monitored using small angle X-ray scattering (SAXS) and nitrogen gas adsorption (N2GA) techniques. No obvious change observed in the characteristic peaks of SAXS intensity curves during consolidation suggests that the intra-particle structure of the clay, including interlayer spacings, is not affected by mechanical loading, at least up to 4 MPa. The N2GA test results show that the volume of inter-particle pores inside the aggregates does decrease gradually as the compression proceeds, which is accompanied by a gradual reduction in specific surface area, probably due to the rearrangement of the particles composing the aggregates. The inter-particle pores are compressed as a whole during consolidation instead of the progressive collapse in an ordered manner, from the larger to the smaller. By comparing the pore-size distributions of illite-dominant clay obtained by MIP (mercury intrusion porosimetry) and N2GA techniques, it is found that the shapes of the two distributions in the common measurement range are obviously not matched, essentially due to the sequential nature of the drying and wetting processes. While filling the research gap in the evolution of intra-aggregate pores during consolidation, this study also shows that the N2GA technique and SAXS measurement used in conjunction with each other appear as a powerful approach for clay nano-pores identification. [ABSTRACT FROM AUTHOR]
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- 2024
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5. Perceptions of infection control professionals toward electronic surveillance software supporting inpatient infections: A mixed methods study.
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Baudet, Alexandre, Brennstuhl, Marie-Jo, Lizon, Julie, Regad, Marie, Thilly, Nathalie, Demoré, Béatrice, and Florentin, Arnaud
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- 2024
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6. Effects of temperature on the performance of Palaemon serratus (Pennant, 1777) larvae from winter and summer laying.
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Baudet, Jean-Baptiste, Xuereb, Benoit, Danger, Michael, Felten, Vincent, Duflot, Aurélie, Poret, Agnès, Le Foll, Frank, and Coulaud, Romain
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TEMPERATURE effect , *LARVAE , *METAMORPHOSIS , *BIOMASS , *SEASONS - Abstract
In the common prawn, Palaemon serratus (Pennant, 1777), the females release larvae twice a year (winter and summer layings). We investigated seasonal differences in larval phenotypes and their consequences on larval performance. We measured the biomass and carbon (C) and nitrogen (N) content (proxy of lipid and protein reserves) at hatching of larvae laid by 6 winter and 6 summer females collected on the coast of Seine-Maritime (France). We incubated these larvae at 3 temperatures (12, 16, and 20 °C) and quantified the effects of temperature and season on survival, development time, biomass, and C and N content and ratio at metamorphosis. At hatching, winter larvae were larger than summer larvae, but their C/N was similar. Development time increased with decreasing temperature, with no seasonal difference. Within the same clutch, the longer the development time, the greater the weight of the larvae, without affecting their C/N ratio. Seasonal differences in maternal per offspring investment were not as pronounced as expected. Surprisingly, the summer larvae survived and grew better than the winter ones, at least at 16 and 20 °C. • We studied the phenotypes of winter and summer larvae of Palaemon serratus and their performance. • At hatching, winter larvae were larger than summer larvae, but their lipid reserves were similar. • Seasonal difference in larval biomass at hatching had little effect on larval performance. • At a given temperature, the longer the larval development, the larger the juveniles. • Lipid reserves of juveniles depend on temperature and season but not on the larval development time. [ABSTRACT FROM AUTHOR]
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- 2024
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7. Landscape of ice and fire – uniquely well-preserved Scots pine trunks reveal forest fires near the retreating Weichselian ice margin.
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Klaminder, Jonatan, Fassl, Magdalena, Baudet, Marlène, Östlund, Lars, Linderholm, Johan, and Zale, Rolf
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- 2024
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8. Combined effects of temperature and diet on the performance of larvae produced by young and old Palaemon serratus females.
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Baudet, Jean-Baptiste, Xuereb, Benoît, Schaal, Gauthier, Rollin, Marc, Poret, Agnès, Jeunet, Léa, Jaffrézic, Enora, Duflot, Aurélie, Charles, Thibault, Le Foll, Frank, and Coulaud, Romain
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LARVAE , *ESSENTIAL fatty acids , *TEMPERATURE effect , *DIET , *FATTY acids , *FOOD quality , *LARVAL dispersal - Abstract
Seasonal variations in environmental conditions determine the success of decapod larval development, and females transmit more energy in sub-optimal conditions to maximise the fitness of their offspring. The objective of this study was to focus on the combined effects of temperature (14, 18 and 22 °C) and food quality on the performance of larvae produced by 5 young (0+) and 5 old (I+) Palaemon serratus females. We prepared 3 diets based on Artemia, in decreasing order of total fatty acid content: freshly hatched nauplii (N), unenriched metanauplii (M) and metanauplii enriched with a mixture of microalgae (ME). At hatching, the larvae produced by I+ females had a higher biomass but a similar fatty acid concentration to those produced by 0+ females. Larvae survived better and developed relatively faster as temperature increased, and the longer they waited to metamorphose, the greater their weight at metamorphosis. These performances were diet-dependent, with more survival and more growth in less time with diet N than with the other two. Larvae from I+ females performed better than those from 0+ females, especially under the most stressful conditions. The greater biomass of the larvae of I+ females seems to have enabled them to follow a shorter, and therefore faster, development path than those of 0+ females. The larvae's diet also had an impact on post-metamorphic composition: larvae eating a diet richer in fatty acids produced richer juveniles and those eating a poorer diet produced juveniles with slightly more essential fatty acids. This study supports the high plasticity of caridean shrimp larval development and the importance of maternal effects on the fitness of offspring. • Temperature, prey quality and maternal effects affect the performance of Palaemon serratus larvae. • Older females produced larger larvae which increased their survival under stressful conditions. • The larvae survived better and developed relatively faster as the temperature increased. • The larvae plastically delayed their metamorphosis and reduced their growth in the face of stress. • Larval performance increased with total fatty acid concentration in Artemia , higher in nauplii. [ABSTRACT FROM AUTHOR]
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- 2024
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9. Small-strain shear stiffness anisotropy of a saturated clayey loess.
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Zuo, Lu, Xu, Ling, Baudet, Béatrice Anne, Gao, Chongyang, and Huang, Chuang
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LOESS , *ANISOTROPY , *MODULUS of rigidity , *SOIL structure , *COMPACTING - Abstract
The mechanical behaviour of clayey loess is strongly affected by the soil structure, but although anisotropy has been identified in loess by some, the anisotropy of small-strain behaviour is rarely reported. This paper presents an experimental study on the inherent and stress-induced small-strain shear stiffness anisotropy of a clayey loess from China. Both undisturbed and reconstituted specimens were tested with bender elements under isotropic compression and shearing conditions. Under an isotropic stress state, an inherent anisotropy was found for undisturbed specimens, while the reconstituted specimens prepared by moist tamping behaved isotropically. During shearing, the ratio of horizontal to vertical shear moduli of the undisturbed specimens decreased due to both an increase of stress anisotropy and the destruction of the intact structure. In contrast, the stiffness ratio of the reconstituted specimens only decreased due to stress anisotropy and it became more anisotropic at the critical state. This study reveals the influence of intact structure and inherent anisotropy on the behaviour of loess soils, which cannot be reproduced by compaction, thus highlighting the importance of characterising the undisturbed loess. [ABSTRACT FROM AUTHOR]
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- 2024
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10. Anorogenic plutonism in the West Congo Belt of the Democratic Republic of Congo further supports early Tonian continental rifting in the Congo – São Francisco palaeocontinent.
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Tack, Luc, Fernandez-Alonso, Max, Baudet, Daniel, De Grave, Johan, Nseka-Mbemba, Patrick, and Pedrosa-Soares, Antonio Carlos
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THRUST belts (Geology) , *IGNEOUS intrusions , *GEOLOGICAL maps , *ULTRABASIC rocks , *RIFTS (Geology) , *CONTINENTS , *MAGMATISM - Abstract
Our paper presents an updated geological map and simplified lithostratigraphy of the West Congo Belt (WCB) in the Democratic Republic of Congo. The WCB holds a key position for understanding the construction of Gondwana and constitutes a larger orogenic system along with its Brazilian counterpart (i.e. Araçuaí orogen). Our current contribution focuses on a newly found and mapped early Tonian small-sized (6 × 4 km) plutonic body (so-called "Shinkakasa intrusive complex") located in the westernmost amphibolite facies tectono-metamorphic domain of the West Congo fold-and-thrust belt. The complex is hosted by the Kimeza gneissic basement. Our fieldwork, samples and a new geological map provide a solid and updated geological background for more in-depth future work and proves essential for reinterpreting the broader tectonic framework. Our findings show that the Shinkakasa complex comprises felsic, intermediate and mafic to ultramafic rocks displaying mixing-mingling features and well-preserved igneous textures with only limited Panafrican metamorphic and tectonic overprint. According to previously published zircon U–Pb (SHRIMP) data, the Shinkakasa magmatism lasted from 911 Ma to 869 Ma and the complex was hosted by the 2069 Ma Kimeza gneissic basement. We compare the Shinkakasa complex with other small-sized intrusive complexes of the WCB, emplaced in a similar geological setting, such as the contemporaneous Mayumba bimodal complex of southwestern Gabon. This comparison is extended across the Atlantic Ocean to the recently studied early Tonian Salto da Divisa complex of southeastern Brazil. In the African counterpart we correlate the plutonic complexes to the early Tonian eastward arcuate (sub)volcanic bimodal successions (Seke-Banza Group of the DRC) largely exposed along trend of the orogen over a length of ca. 550 km in the greenschist facies domain of the WCB. Finally, we give an updated account of the early Tonian emplacement ages and lithostratigraphic terminology of the multiple bodies constituting this arcuate structure. Our data show that i) the vast majority of the anorogenic magmatism testifying for the aborted early Tonian continental rifting of the Congo - Sao Francisco palaeocontinent is exposed in the African counterpart, and ii) the extent of the (sub)volcanic successions largely predominates compared to the small-sized intrusive bodies such as the Shinkakasa complex. • 911-869 Ma Shinkakasa intrusive complex, Democratic Republic of Congo; • anorogenic felsic, intermediate and mafic to ultramafic magmatism; • continental rifting of the Rhyacian-Orosirian Congo – São Francisco palaeocontinent; • 2069 Ma Boma gneiss-migmatite basement block; • update of the early Tonian magmatism of African part of Araçuai – West Congo Orogenic System (AWCO). [ABSTRACT FROM AUTHOR]
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- 2024
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11. BRCA1 Intragenic Duplication Combined with a Likely Pathogenic TP53 Variant in a Patient with Triple-Negative Breast Cancer: Clinical Risk and Management.
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Ea, Vuthy, Berthozat, Claudine, Dreyfus, Hélène, Legrand, Clémentine, Rousselet, Estelle, Peysselon, Magalie, Baudet, Laura, Martinez, Guillaume, Coutton, Charles, and Bidart, Marie
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TRIPLE-negative breast cancer , *BREAST , *BRCA genes , *DISEASE risk factors , *OVARIAN cancer , *LI-Fraumeni syndrome - Abstract
For patients with hereditary breast and ovarian cancer, the probability of carrying two pathogenic variants (PVs) in dominant cancer-predisposing genes is rare. Using targeted next-generation sequencing (NGS), we investigated a 49-year-old Caucasian woman who developed a highly aggressive breast tumor. Our analyses identified an intragenic germline heterozygous duplication in BRCA1 with an additional likely PV in the TP53 gene. The BRCA1 variant was confirmed by multiplex ligation probe amplification (MLPA), and genomic breakpoints were characterized at the nucleotide level (c.135-2578_442-1104dup). mRNA extracted from lymphocytes was amplified by RT-PCR and then Sanger sequenced, revealing a tandem duplication r.135_441dup; p.(Gln148Ilefs*20). This duplication results in the synthesis of a truncated and, most likely, nonfunctional protein. Following functional studies, the TP53 exon 5 c.472C > T; p.(Arg158Cys) missense variant was classified as likely pathogenic by the Li-Fraumeni Syndrome (LFS) working group. This type of unexpected association will be increasingly identified in the future, with the switch from targeted BRCA sequencing to hereditary breast and ovarian cancer (HBOC) panel sequencing, raising the question of how these patients should be managed. It is therefore important to record and investigate these rare double-heterozygous genotypes. [ABSTRACT FROM AUTHOR]
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- 2024
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12. Feasibility of CD19 CAR T-cell therapy in patients with cardiac lymphoma.
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Kuipers, Maria T., Spanjaart, Anne M., Bonifazi, Francesca, diBlasi, Roberta, Zinzani, Pier L., Thieblemont, Catherine, Baudet, Mathilde, Biemond, Bart J., Kok, Wouter E. M., and Kersten, Marie J.
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CARDIAC patients , *ARRHYTHMIA , *T cells , *CD19 antigen , *LYMPHOMAS , *DIFFUSE large B-cell lymphomas , *CARDIAC magnetic resonance imaging - Abstract
This document discusses the feasibility of using CD19 CAR T-cell therapy in patients with cardiac lymphoma. The article highlights that cardiac non-Hodgkin lymphoma is not well-represented in medical literature, and the exact incidence is unknown. The authors present three cases of patients with cardiac lymphoma who received CAR T-cell therapy. The first two patients had successful outcomes with no significant cardiac events, while the third patient experienced severe cardiotoxicity after CAR T infusion. The authors suggest that a multidisciplinary approach and preventive measures for severe cytokine release syndrome are important in optimizing the safety and efficacy of CAR T-cell therapy in patients with cardiac lymphoma. [Extracted from the article]
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- 2024
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13. Lost in transformation: comparative analysis of healthcare provision dynamics within urban systems of European Russia and France.
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Gunko, Maria, Conti, Benoit, Sheludkov, Alexander, Baudet-Michel, Sophie, and Novkunskaya, Anastasia
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URBANIZATION , *HEALTH care reform , *NEW public management , *CITIES & towns , *HOSPITAL supplies - Abstract
Since the 1990s, many countries have implemented healthcare reforms underlined by New Public Management principles and technological transformations. Although studies have examined these reforms from different viewpoints, the spatial implications of healthcare reforms have received limited attention. Scholarship focused predominantly on regional variations of healthcare provision overlooking the sharp contrasts between cities where most healthcare facilities are de facto located. Addressing this research gap, we investigate the long-term dynamics of healthcare provision on the urban level, tracing the differences (if any) between cities of different sizes and administrative statuses. The study adopts a comparative approach. We draw our data from two countries: France and Russia (mainland France and European Russia). Findings indicate that, despite some variations, healthcare reforms in both countries follow similar paths, resulting in fewer hospital beds that have been partially replaced by places in day hospitals. At the same time, we also observe diverging country-specific trends in terms of redistribution of healthcare provision. In France, some cities completely lost their hospital equipment but those cities that remained equipped tend to a uniform distribution. In European Russia, on the contrary, all cities remain equipped but there is a drastic polarization depending on size and administrative status. [ABSTRACT FROM AUTHOR]
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- 2024
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14. ANCA-associated scleritis: impact of ANCA on presentation, response to therapy and outcome.
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Perray, Laura, Nguyen, Yann, Refregiers, Gaëlle Clavel, Chazal, Thibaud, Héron, Emmanuel, Pouchelon, Clara, Dunogué, Bertrand, Costedoat-Chalumeau, Nathalie, Murarasu, Anne, Régent, Alexis, Puéchal, Xavier, Thoreau, Benjamin, Lifermann, François, Graveleau, Julie, Hié, Miguel, Froissart, Antoine, Baudet, Antoine, Deroux, Alban, Lavigne, Christian, and Puigrenier, Sébastien
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RESEARCH , *C-reactive protein , *GLUCOCORTICOIDS , *RITUXIMAB , *DISEASE progression , *CONFIDENCE intervals , *ANTINEUTROPHIL cytoplasmic antibodies , *SCLERA , *ACQUISITION of data , *RETROSPECTIVE studies , *CASE-control method , *TERTIARY care , *TREATMENT effectiveness , *RISK assessment , *MEDICAL records , *DESCRIPTIVE statistics , *EYE diseases , *LONGITUDINAL method , *SYMPTOMS - Abstract
Objectives To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests. Methods This retrospective multicentre case–control study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data from patients with scleritis without any systemic manifestation and with positive ANCA results were compared with those of a control group of patients with idiopathic scleritis with negative ANCA tests. Results A total of 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022 were included. The median follow-up was 28 months (IQR 10–60). The median age at diagnosis was 48 years (IQR 33–60) and 75% were females. Scleromalacia was more frequent in ANCA-positive patients (P = 0.027) and 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% vs 34%; P < 0.001), and rituximab (P = 0.03) and had a lower remission rate after the first- and second-line treatment. Systemic ANCA-associated vasculitis (AAV) occurred in 30.7% of patients with PR3- or MPO-ANCA, after a median interval of 30 months (IQR 16.3–44). Increased CRP >5 mg/l at diagnosis was the only significant risk factor of progression to systemic AAV [adjusted hazard ratio 5.85 (95% CI 1.10, 31.01), P = 0.038]. Conclusion Isolated ANCA-associated scleritis is mostly anterior scleritis with a higher risk of scleromalacia than ANCA-negative idiopathic scleritis and is more often difficult to treat. One-third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV. [ABSTRACT FROM AUTHOR]
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- 2024
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15. Critical state analysis of two compacted filtered iron ore tailings with different gradings and mineralogy at different stages of treatment.
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Consoli, Nilo Cesar, Silva, João Paulo Sousa, Wagner, Alexia Cindy, Carvalho, João Vítor de Azambuja, Baudet, Beatrice Anne, Coop, Matthew Richard, Scheuermann Filho, Hugo Carlos, Carvalho, Inácio, de Sousa, Gustavo Marçal, and Cacciari, Pedro Pazzoto
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IRON ores , *MINERALOGY , *SOIL mechanics , *CRITICAL analysis , *AEROSPACE planes , *IRON mining , *COIN grading - Abstract
Slurry tailings storage in large impoundments has been largely used worldwide for a long time, as their cost is very competitive. However, recent disasters have brought to light the need to better comprehend the mechanics of the materials stored and to search for disposal alternatives to overcome the drawbacks. One possibility is the filtered tailings disposal (dry stacking) which requires a better understanding of the material's response in a dewatered (through filtration) and compacted condition. This paper compares two tailings from the same beneficiation (treatment) plant with different gradings and mineralogy, related to the beneficial processes they undergo. A series of triaxial tests comprising isotropic compression without shearing specimens, as well as isotropic compression followed by drained (CID) and undrained (CIU) shearing, and K-compression followed by undrained (CKU) shearing specimens were conducted over a range of confining pressures and initial compaction degrees. The experimental program allowed the evaluation of convergence for normal compression lines (NCLs) and the analysis under the light of critical state soil mechanics for the stress–strain response of the tested materials. The research outcomes show that changes in iron ore tailings gradings due to different production processes and the use of different compaction degrees had an influence on its behavior (compression and shearing) at lower stress levels, while at higher stresses levels, this difference is erased and there is a convergence for unique and parallels NCL and CSL on ν–ln p′ plane with a spacing of 2.71. On the p′–q plane both tailings showed a unique and similar CSL. [ABSTRACT FROM AUTHOR]
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- 2024
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16. Les hyperéosinophilies et syndromes hyperéosinophiliques : caractéristiques des 550 premiers patients inclus dans la cohorte COHESion, une cohorte nationale multicentrique.
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Lefèvre, G., Bleuse, S., Ledoult, E., Panel, K., Abou Chahla, W., Elenga Koanga, J.D., Schwarb, L., Outh, R., Terriou, L., Dion, J., Dossier, A., Moulis, G., Abisror, N., Limal, N., Baudet, A., Slama, B., Lioger, B., Viallard, J.F., Machelart, I., and Trefond, L.
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Les syndromes hyperéosinophiliques (SHE) et les hyperéosinophilies (HE) sont un groupe hétérogène de maladies, dont les étiologies peuvent être clonales, réactionnelles (secondaires) ou idiopathiques. À ce jour, seules des études rétrospectives de taille et/ou de durée de suivi limitées ont décrit les caractéristiques des patients avec les différentes formes d'HE ou SHE, et principalement dans des centres experts. Nous décrivons ici la mise en place d'une cohorte nationale associant des centres experts du CEREO, et des services de différentes spécialités au sein d'hôpitaux universitaires ou généraux, et les principales caractéristiques des patients inclus. L'étude COHESion est une cohorte nationale prospective multicentrique (n > 50 centres actifs), multidisciplinaire, recrutant depuis le 1er mai 2019 à la fois des adultes et des enfants, déjà suivis dans le centre ou nouvellement diagnostiqués. Les critères d'éligibilité englobaient le spectre complet des HE/SHE, notamment les HE/SHE réactionnels (HE/SHE-R) secondaires à une infection notamment parasitaire, une hypersensibilité médicamenteuse retardée (HSR), une maladie auto-immune ou inflammatoire, un cancer solide ou une hémopathie, mais aussi le variant lymphoïde (HE/SHE-L), les HE/SHE clonaux ou « myéloïdes » (HE/SHE-M), les SHE idiopathiques (SHE-I), les HE asymptomatiques de signification indéterminée (HE-US), les situations de chevauchements entre SHE et la maladie associée aux IgG4, ou la granulomatose éosinophilique avec polyangéite (GEPA) ANCA-négative (selon les critères adaptés de la classification ACR/EULAR 2022 avec au moins un asthme, et une vascularite clinique et/ou histologiquement prouvée). Chez les patients atteints de SHE-I, les profils évolutifs ont été classés ainsi : (i) profil de « poussée unique » lorsqu'une seule poussée est survenue, traitée ou non, sans rechute ultérieure, (ii) profil de poussées récurrentes avec rémissions lorsqu'il y avait au moins une fois un intervalle de 6 mois sans symptômes entre deux poussées, et (iii) profil de « maladie chronique persistante » quand une corticothérapie au long cours était nécessaire pour éviter les rechutes précoces. Toutes les données ont été saisies et analysées de manière centralisée par les chargé(e)s de projets et attaché(e)s de recherche clinique du CEREO. Après 54 mois d'activité, 779 patients ont été inclus dans des hôpitaux généraux (20 %), des hôpitaux universitaires (46 %) ou dans un des centres experts du CEREO (34 %). Au moment de l'analyse, 550 cas étaient disponibles pour une analyse centralisée (âge moyen (±ET) : 56 ± 18 ans, 42 % de patientes). Les diagnostics étaient les suivants : SHE idiopathiques (n = 258, 47 %), HE/SHE-R (n = 87, 16 %), HE-US (n = 80, 15 %), HE/SHE-M (n = 40, 7 %), HE/SHE-L (n = 34, 6 %). Parmi les patients atteints de HE/SHE-R (n = 87), les maladies sous-jacentes ou associées étaient une HSR (23 %), une parasitose (22 %), une hémopathie lymphoïde (18 %), un cancer solide (10 %), une maladie inflammatoire chronique (8 %), une mastocytose systémique (6 %) ou d'autres causes (13 %). Par ailleurs, certains patients remplissaient également les critères de la maladie associée aux IgG4 (n = 13, 2 %) ou de GEPA ANCA-négative (n = 38, 7 %). À la dernière visite, les profils évolutifs des 258 patients atteints de SHE-I étaient les suivants : n = 42 (16 %) poussée unique, n = 73 (28 %) formes récurrentes et n = 119 (46 %) maladies chroniques persistantes. Enfin, 24 (9 %) cas restaient non classés en raison d'un recul insuffisant suite au diagnostic initial. La cohorte COHESion est la première étude prospective multicentrique et multidisciplinaire de dimension nationale, collectant des données sur les patients atteints de HE et SHE. Cette cohorte permettra de décrire en détail les différentes formes de SHE, les atteintes d'organes, leur pronostic, et le bénéfice des nouvelles thérapies ciblées disponibles dans les différents variants de HE et SHE. Cette cohorte confirme déjà que les patients atteints de SHE idiopathiques présentent des profils évolutifs différents, suggérant des mécanismes physiopathologiques distincts, et la nécessité de stratégies thérapeutiques personnalisées. [ABSTRACT FROM AUTHOR]
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- 2024
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17. Development of a scoring system to define lysosomal diseases.
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Yuzyuk, Tatiana, Wilk, Matheus Vernet Machado Bressan, Goldstein, Jenny, Groopman, Emily, Mohan, Shruthi, Waddell, Amber, Fernandez, Raquel, Chen, Hongjie, Bali, Deeksha, Baudet, Heather, Clarke, Lorne, Hung, Christina, Mao, Rong, Craigen, William, and Pinto e Vairo, Filippo
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- 2024
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18. Applying phenotypic evidence to support the molecular diagnosis of inborn errors of metabolism (IEMS) across diverse clinical contexts: The ClinGen IEM variant curation expert panel (VCEP) experience.
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Groopman, Emily, Goldstein, Jenny, Dickson, Alexa, Zastrow, Diane, Mohan, Shruthi, Thomas-Wilson, Amanda, Caldovic, Ljubica, Kyle, Emily, Yuzyuk, Tatiana, De Biase, Irene, Simpson, Kara, Kanavy, Dona, Spector, Elaine, Pasquali, Marzia, Rehder, Catherine, Stergachis, Andrew, Whirl-Carlo, Michelle, Baudet, Heather, Hung, Christina, and Braverman, Nancy E.
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INBORN errors of metabolism , *DIAGNOSTIC errors , *MOLECULAR diagnosis , *PHENOTYPES - Published
- 2024
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19. Investigation of hydrothermal activity in the South West Indian ridge region using Ra isotopes and 227Ac as tracers.
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Léon, Morgane, van Beek, Pieter, Sanial, Virginie, Baudet, Corentin, Charette, Matthew A., Souhaut, Marc, Vivier, Frédéric, Kestenare, Elodie, Jeandel, Catherine, and Planquette, Hélène
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RADIUM isotopes , *ISOTOPES , *CHEMICAL systems , *HYDROTHERMAL vents , *CHEMICAL elements - Abstract
• Short-lived Ra isotopes highlight the presence of a hydrothermal activity on the SWIR. • The hydrothermal system is located at a distance < 30 km of the investigated stations. • A strong vertical mixing is observed (Kz from 38 up to 149 cm2 s−1) • A large dFe vertical flux from 552 up to1173 nmol m−2 d-1 are estimated. Hydrothermal vents have been shown to be important vectors for various chemical elements into the ocean. However, both the intensity of the chemical fluxes associated with these systems and the fate of the chemical elements along the plume are still largely overlooked. At two stations located above the South West Indian Ridge (SWIR), we investigate the distributions of the Ra quartet (223Ra, 224Ra, 226Ra and 228Ra) and 227Ac that have been used as tracers of hydrothermal activity. While the vertical distributions of 226Ra, 228Ra and 227Ac do not show a clear enrichment at depth, unusual signatures of excess 223Ra and 224Ra near the seafloor are attributed to the presence of a hydrothermal activity. The discrepancy observed between the different isotopes is attributed to different chemical reactivity when seawater circulates within the crust and/or to different regeneration rates within the fluid. A 1D diffusion model applied to the vertical profiles of short-lived Ra isotopes provided an estimation of the vertical eddy diffusivity coefficients (K Z) between 38 cm2 s−1 and 149 cm2 s−1. These high values suggest strong mixing likely favored by the complex bathymetry in the region. By combining these K Z with the vertical gradient of dissolved Fe (dFe), we estimate a vertical flux of dFe that ranges from 139 to 1173 nmol m−2 d-1. These results confirm that low-expansion-rate ridges could be significant sources of dFe to the deep ocean. [ABSTRACT FROM AUTHOR]
- Published
- 2024
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