The IFMIF-EVEDA accelerator beam dump design

The IFMIF-EVEDA accelerator will be a 9 MeV, 125 mA cw deuteron accelerator prototype for verifying the validity of the 40 MeV accelerator design for IFMIF. A beam dump designed for maximum power of 1.12 MW will be used to stop the beam at the accelerator exit. The conceptual design for the IFMIF-EVEDA accelerator beam dump is based on a conical beam stop made of OFE copper. The cooling system uses an axial high velocity flow of water pressurized up to 3.4 × 105 Pa to avoid boiling. The design has been shown to be compliant with ASME mechanical design rules under nominal full power conditions. A sensitivity analysis has been performed to take into account the possible margin on the beam properties at the beam dump entrance. This analysis together with the study of the maintenance issues and the mounting and dismounting operations has led to the complete design definition

Síndrome urémico hemolítico: eco-epidemiología del enemigo que afecta la seguridad alimentaria

Escherichia coli verocitotoxigénico (VTEC) es un patógeno emergente asociado a casos de diarrea, colitis hemorrágica y síndrome urémico hemolítico (SUH), del cual Argentina presenta el registro más alto de casos a nivel mundial. No existe tratamiento específico para el SUH, por lo que son fundamentales las estrategias de control y prevención. Los estudios se han centralizado en cepas del serotipo O157:H7, pero las infecciones asociadas a VTEC no-O157 tienen creciente importancia y representan nuevos desafíos para el diagnóstico y el control de VTEC. En este trabajo se evaluó el rol de distintas especies animales, medio ambiente y alimentos en la epidemiología de VTEC, se caracterizó la variabilidad genética y la virulencia de las cepas, y se estudiaron medidas de control. Las metodologías comprenden técnicas microbiológicas y de biología molecular. Los resultados confirman la amplia distribución de las cepas VTEC, demuestran su gran diversidad genética y la presencia de factores de virulencia asociados con enfermedad en el hombre. Las cepas estudiadas mostraron alta capacidad para sobrevivir en el ambiente. La exhaustiva caracterización de las cepas resalta el alto riesgo para la salud pública que representan.Trabajo presentado por el Centro de Investigación Veterinaria de Tandil (CIVETAN)

Economic Valuation of Health Care Services in Public Health Systems: A Study about Willingness to Pay (WTP) for Nursing Consultations

Background: Identifying the economic value assigned by users to a particular health service is of principal interest in planning the service. The aim of this study was to evaluate the perception of economic value of nursing consultation in primary care (PC) by its users. Methods and Results: Economic study using contingent valuation methodology. A total of 662 users of nursing consultation from 23 health centers were included. Data on demographic and socioeconomic characteristics, health needs, pattern of usage, and satisfaction with provided service were compiled. The validity of the response was evaluated by an explanatory mixed-effects multilevel model in order to assess the factors associated with the response according to the welfare theory. Response reliability was also evaluated. Subjects included in the study indicated an average Willingness to Pay (WTP) of €14.4 (CI 95%: €13.2–15.5; median €10) and an average Willingness to Accept [Compensation] (WTA) of €20.9 (CI 95%: €19.6–22.2; median €20). Average area income, personal income, consultation duration, home visit, and education level correlated with greater WTP. Women and older subjects showed lower WTP. Fixed parameters explained 8.41% of the residual variability, and response clustering in different health centers explained 4–6% of the total variability. The influence of income on WTP was different in each center. The responses for WTP and WTA in a subgroup of subjects were consistent when reassessed after 2 weeks (intraclass correlation coefficients 0.952 and 0.893, respectively). Conclusions: The economic value of nursing services provided within PC in a public health system is clearly perceived by its user. The perception of this value is influenced by socioeconomic and demographic characteristics of the subjects and their environment, and by the unique characteristics of the evaluated service. The method of contingent valuation is useful for making explicit this perception of value of health services

Novel mutations expand the clinical spectrum of DYNC1H1-associated spinal muscular atrophy

OBJECTIVE To expand the clinical phenotype of autosomal dominant congenital spinal muscular atrophy with lower extremity predominance (SMA-LED) due to mutations in the dynein, cytoplasmic 1, heavy chain 1 (DYNC1H1) gene. METHODS Patients with a phenotype suggestive of a motor, non-length-dependent neuronopathy predominantly affecting the lower limbs were identified at participating neuromuscular centers and referred for targeted sequencing of DYNC1H1. RESULTS We report a cohort of 30 cases of SMA-LED from 16 families, carrying mutations in the tail and motor domains of DYNC1H1, including 10 novel mutations. These patients are characterized by congenital or childhood-onset lower limb wasting and weakness frequently associated with cognitive impairment. The clinical severity is variable, ranging from generalized arthrogryposis and inability to ambulate to exclusive and mild lower limb weakness. In many individuals with cognitive impairment (9/30 had cognitive impairment) who underwent brain MRI, there was an underlying structural malformation resulting in polymicrogyric appearance. The lower limb muscle MRI shows a distinctive pattern suggestive of denervation characterized by sparing and relative hypertrophy of the adductor longus and semitendinosus muscles at the thigh level, and diffuse involvement with relative sparing of the anterior-medial muscles at the calf level. Proximal muscle histopathology did not always show classic neurogenic features. CONCLUSION Our report expands the clinical spectrum of DYNC1H1-related SMA-LED to include generalized arthrogryposis. In addition, we report that the neurogenic peripheral pathology and the CNS neuronal migration defects are often associated, reinforcing the importance of DYNC1H1 in both central and peripheral neuronal functions

Significance of Degree of HLA Disparity Using T-cell Replete Peripheral Blood Stem Cells from Haploidentical Donors with Posttransplantation Cyclophosphamide in AML in First Complete Hematologic Remission: A Study of the Acute Leukemia Working Party of the EBMT

Availability of haploidentical donors has broadened utilization of allogeneic hematopoietic cell transplantation (allo-HCT). Peripheral blood stem cells (PBSC) are being used with increased frequency in haploidentical allo-HCT. We evaluated extent of HLA disparity (2-3/8 versus 4/8 HLA antigen mismatches) on post-allograft outcomes when using T-cell replete PBSC from haploidentical donors for acute myeloid leukemia in first complete remission. Primary objectives entailed assessing cumulative incidence of grade 2-4 acute graft-versus-host disease (GVHD) and chronic GVHD (any grade). A total of 645 patients received a haploidentical allo-HCT from a donor with either 2-3 of 8 HLA antigen mismatches (n = 180) or with 4 of 8 HLA antigen mismatches (n = 465). Presence of 2-3 of 8 versus 4 of 8 HLA mismatches did not affect the incidence of acute GVHD (grade 2-4) and chronic GVHD (any grade). Overall survival (OS), leukemia-free survival (LFS) relapse incidence (RI), nonrelapse mortality and the composite endpoint of GVHD-free relapse-free survival were also similar among the groups. Pertaining to HLA-B leader matching effect, our analysis did not discern any difference in aforementioned post-allograft outcomes for this variable. However, in univariate analysis, absence of an antigen mismatch in HLA-DPB1 showed a trend for better OS. Notwithstanding inherent limitations associated with registry data, our results did not show an advantage of selecting a haploidentical donor with 2-3 of 8 HLA antigen mismatches over one with 4 of 8 HLA antigen mismatches when using PBSC as the cell source. Adverse cytogenetics remains a major adverse determinant of inferior OS and LFS and a higher RI. Using reduced-intensity conditioning yielded worse OS and LFS

Sedimentation and biomineralization processes in Las Eras Lake (Coca-Olmedo wetland)

La Laguna de Las Eras es uno de los pequeños y someros cuerpos de agua, que forman el humedal de la zona Coca-Olmedo (cuenca del Duero), caracterizado por su elevada alcalinidad, lo que constituye un rasgo singular dentro de Europa. La laguna presenta una salmuera de tipo Na-Mg-ClSO4 y su superficie está colonizada por tapices microbianos, donde se desarrollan estructuras sedimentarias inducidas por los microorganismos (MISS). Se reconocen diversos minerales autigénicos asociados a los tapices: thenardita, hidromagnesita,sulfatos y fosfatos magnésicos, azufre y halita. Junto a éstos destacan, por ser carbonatos atípicos en Europa, natrón y trona. El estudio petrográfico de los precipitados revela que éstos guardan una estrecha relación con las estructuras microbianas, sugiriendo cierta influencia de los microorganismos en la precipitación mineral.Los tapices microbianos de la laguna de Las Eras constituyen buenos análogos para comprender los procesos geobiológicos y ahondar en la reconstrucción paleoambiental de los lagos alcalinos que han existido desde el Arcaico.Las Eras Lake is one of the small and shallow bodies of water, which form the highly alkaline Coca-Olmedo wetland (Duero Basin), a unique feature within Europe. The lake brine is a Na-Mg-Cl-SO4 type. The lake hosts benthic microbial mats and its surface shows microbially influenced sedimentary structures (MISS). Associated with the microbial mats, several authigenic minerals are recognized such as thenardite, hydromagnesite, magnesium-bearing sulphate and phosphate, halite and sulphur.Among these we highlight the occurrence of natron and trona because the sodium-bearing carbonates are uncommon in the european region. The scanning electron microscopy study reveals that the minerals are closely related to microbial structures,suggesting some influence of microorganisms in the mineral precipitation. Recent microbial mats as those hosted in Las Eras Lake are good analogues for understanding geobiological processes. Knowledge of these processes provides a model for paleoenvironmental reconstruction of alkaline endorheic lakes that have existed since the Archean.Depto. de Biodiversidad, Ecología y EvoluciónFac. de Ciencias BiológicasTRUEMinisterio de Economía y Competitividad (MINECO)pu