[La microstructure 3D des matériaux polycristallins vue sous la lumière synchrotron]

International audienceSynchrotron radiation X-ray imaging and diffraction techniques offer new possibilities for non-destructive bulk characterization of polycrystalline materials. Minute changes in electron density (different crystallographic phases, cracks, porosities) can be detected using 3D imaging modes exploiting Fresnel diffraction and the coherence properties of third generation synchrotron beams. X-ray diffraction contrast tomography, a technique based on Bragg diffraction imaging, provides access to the 3D shape, orientation and elastic strain state of the individual grains from polycrystalline sample volumes containing several hundred up to a few thousand grains. Combining both imaging modalities allows a comprehensive description of the microstructure of the material at the micrometer length scale. Repeated observations during (interrupted) mechanical tests provide unprecedented insight into crystallographic and grain microstructure related aspects of polycrystal deformation and degradation mechanisms in materials, fulfilling some conditions on grain size and deformation state.Les techniques d'imagerie et de diffraction au rayonnement synchrotron offrent de nouvelles possibilités pour la caractérisation tridimensionnelle et non destructive des matériaux polycristallins. De faibles variations de densité électronique (phases secondaires, fissures, porosités) peuvent êtres détectées grâce à des modes d'imagerie qui exploitent la diffraction de Fresnel ainsi que la cohérence des faisceaux issus des sources synchrotron de troisième génération. La tomographie par contraste de diffraction, autre technique d'imagerie 3D basée sur la diffraction de Bragg, donne accès à la forme, l'orientation et l'état de déformation élastique des grains dans des volumes polycristallins contenant jusqu'à mille grains. La combinaison de ces deux modes d'imagerie permet de caractériser des matériaux polycristallins à l'échelle du micron. Des observations répétées lors d'essais mécaniques (interrompus) permettent d'analyser le rôle de la cristallographie locale sur les mécanismes de déformation et de dégradation dans des matériaux polycristallins, respectant certaines conditions sur la taille de grains, et/ou leur état de déformation

Means to an End: An Assessment of the Status-blind Approach to Protecting Undocumented Worker Rights

This article applies the tenets of bureaucratic incorporation theory to an investigation of bureaucratic decision making in labor standards enforcement agencies (LSEAs), as they relate to undocumented workers. Drawing on 25 semistructured interviews with high-level officials in San Jose and Houston, I find that bureaucrats in both cities routinely evade the issue of immigration status during the claims-making process, and directly challenge employers’ attempts to use the undocumented status of their workers to deflect liability. Respondents offer three institutionalized narratives for this approach: (1) to deter employer demand for undocumented labor, (2) the conviction that the protection of undocumented workers is essential to the agency’s ability to regulate industry standards for all workers, and (3) to clearly demarcate the agency’s jurisdictional boundaries to preserve institutional autonomy and scarce resources. Within this context, enforcing the rights of undocumented workers becomes simply an institutional means to an end

Post-transplantation Cyclophosphamide-based Haploidentical Transplantation As Alternative To Matched Sibling Or Unrelated Donor Transplantation For Hodgkin Lymphoma: A Registry Study Of The Lymphoma Working Party Of The European Society For Blood And Marrow Transplantation

Purpose: To compare the outcome of patients with Hodgkin lymphoma who received post-transplantation cyclophosphamide-based haploidentical (HAPLO) allogeneic hematopoietic cell transplantation with the outcome of patients who received conventional HLA-matched sibling donor (SIB) and HLA-matched unrelated donor (MUD). Patients and Methods: We retrospectively evaluated 709 adult patients with Hodgkin lymphoma who were registered in the European Society for Blood and Marrow Transplantation database who received HAPLO (n = 98), SIB (n = 338), or MUD (n = 273) transplantation. Results: Median follow-up of survivors was 29 months. No differences were observed between groups in the incidence of acute graft-versus-host disease (GVHD). HAPLO was associated with a lower risk of chronic GVHD (26%) compared with MUD (41%; P =.04). Cumulative incidence of nonrelapse mortality at 1 year was 17%, 13%, and 21% in HAPLO, SIB, and MUD, respectively, and corresponding 2-year cumulative incidence of relapse or progression was 39%, 49%, and 32%, respectively. On multivariable analysis, relative to SIB, nonrelapse mortality was similar in HAPLO (P =.26) and higher in MUD (P =.003), and risk of relapse was lower in both HAPLO (P =.047) and MUD (P,.001). Two-year overall survival and progression-free survival were 67% and 43% for HAPLO, 71% and 38% for SIB, and 62% and 45% for MUD, respectively. There were no significant differences in overall survival or progression-free survival between HAPLO and SIB or MUD. The rate of the composite end point of extensive chronic GVHD and relapse-free survival was significantly better for HAPLO (40%) compared with SIB (28%; P =.049) and similar to MUD (38%; P =.59). Conclusion: Post-transplantation cyclophosphamide-based HAPLO transplantation results in similar survival outcomes compared with SIB and MUD, which confirms its suitability when no conventional donor is available. Our results also suggest that HAPLO results in a lower risk of chronic GVHD than MUD transplantation

Outcome of hematopoietic cell transplantation for DNA double-strand break repair disorders

Background: Rare DNA breakage repair disorders predispose to infection and lymphoreticular malignancies. Hematopoietic cell transplantation (HCT) is curative, but coadministered chemotherapy or radiotherapy is damaging because of systemic radiosensitivity. We collected HCT outcome data for Nijmegen breakage syndrome, DNA ligase IV deficiency, Cernunnos-XRCC4-like factor (Cernunnos-XLF) deficiency, and ataxia-telangiectasia (AT). Methods: Data from 38 centers worldwide, including indication, donor, conditioning regimen, graft-versus-host disease, and outcome, were analyzed. Conditioning was classified as myeloablative conditioning (MAC) if it contained radiotherapy or alkylators and reduced-intensity conditioning (RIC) if no alkylators and/or 150 mg/m(2) fludarabine or less and 40 mg/kg cyclophosphamide or less were used. Results: Fifty-five new, 14 updated, and 18 previously published patients were analyzed. Median age at HCT was 48 months (range, 1.5-552 months). Twenty-nine patients underwent transplantation for infection, 21 had malignancy, 13 had bone marrow failure, 13 received pre-emptive transplantation, 5 had multiple indications, and 6 had no information. Twenty-two received MAC, 59 received RIC, and 4 were infused; information was unavailable for 2 patients. Seventy-three of 77 patients with DNA ligase IV deficiency, Cernunnos-XLF deficiency, or Nijmegen breakage syndrome received conditioning. Survival was 53 (69%) of 77 and was worse for those receiving MAC than for those receiving RIC (P=.006). Most deaths occurred early after transplantation, suggesting poor tolerance of conditioning. Survival in patients with AT was 25%. Forty-one (49%) of 83 patients experienced acute GvHD, which was less frequent in those receiving RIC compared with those receiving MAC (26/56 [46%] vs 12/21 [57%], P=.45). Median follow-up was 35 months (range, 2-168 months). No secondary malignancies were reported during 15 years of follow-up. Growth and developmental delay remained after HCT; immune-mediated complications resolved. Conclusion: RIC HCT resolves DNA repair disorder associated immunodeficiency. Long-term follow-up is required for secondary malignancy surveillance. Routine HCT for AT is not recommended.Peer reviewe

Long-term follow-up of IPEX syndrome patients after different therapeutic strategies : an international multicenter retrospective study

Background: Immunodysregulation polyendocrinopathy enteropathy x-linked(IPEX) syndrome is a monogenic autoimmune disease caused by FOXP3 mutations. Because it is a rare disease, the natural history and response to treatments, including allogeneic hematopoietic stem cell transplantation (HSCT) and immunosuppression (IS), have not been thoroughly examined. Objective: This analysis sought to evaluate disease onset, progression, and long-term outcome of the 2 main treatments in long-term IPEX survivors. Methods: Clinical histories of 96 patients with a genetically proven IPEX syndrome were collected from 38 institutions worldwide and retrospectively analyzed. To investigate possible factors suitable to predict the outcome, an organ involvement (OI) scoring system was developed. Results: We confirm neonatal onset with enteropathy, type 1 diabetes, and eczema. In addition, we found less common manifestations in delayed onset patients or during disease evolution. There is no correlation between the site of mutation and the disease course or outcome, and the same genotype can present with variable phenotypes. HSCT patients (n = 58) had a median follow-up of 2.7 years (range, 1 week-15 years). Patients receiving chronic IS (n 5 34) had a median follow-up of 4 years (range, 2 months-25 years). The overall survival after HSCT was 73.2% (95% CI, 59.4-83.0) and after IS was 65.1% (95% CI, 62.8-95.8). The pretreatment OI score was the only significant predictor of overall survival after transplant (P = .035) but not under IS. Conclusions: Patients receiving chronic IS were hampered by disease recurrence or complications, impacting long-term.disease-free survival. When performed in patients with a low OI score, HSCT resulted in disease resolution with better quality of life, independent of age, donor source, or conditioning regimen

Olha profe, eu pareço com ela! As Implicações da ERER na Construção de Identidade Racial

A presente pesquisa consiste em um trabalho de conclusão do curso de Pedagogia da Universidade Federal do Rio Grande do Sul. O trabalho, de aspecto reflexivo e analítico, desenvolve-se a partir dos Estudos culturais de perspectiva pós-estruturalista. Tem foco no Ensino Para Relações Étnico-Raciais (ERER) e nas implicações dessa educação para os sujeitos alunos. Para tanto, a pesquisa compreende abordagem qualitativa. Desenvolve-se em torno de um estudo de caso que se potencializou através de análise documental e observação participante, durante o período de estágio obrigatório curricular do curso. Tendo como principal questionamento “como o planejamento/currículo pode contribuir para a construção de identidade racial?”, objetivou-se ao longo do presente trabalho, atentar para as visibilidades conferidas as questões étnico-raciais, e também investigar de que forma os/as alunos/alunas negros/negras se viam (se de fato ocorriam essas visibilidades). Inicialmente, são feitas algumas análises sobre o currículo escolar e, posteriormente sobre a docência. Fundamentada nos conceitos de currículo, identidade e educação para relações étnico-raciais propostos por Tomaz Tadeu da Silva (2001), Jurjo Torres Santome (1995) e Nilma Lino Gomes, Petronilha Beatriz Gonçalves e Silva (2002). O estudo indica que na escola em questão há falta de pertencimento racial e até mesmo resistência dos/das alunos/alunas negros/negras em reconhecerem-se como tais. Entretanto, a equipe diretiva mostrara-se empenhada em modificar esta realidade. As análises apontam ainda a importância existente na ação pedagógica capaz de modificar positivamente a vida das crianças e adolescentes