5,025 research outputs found

    Conceptual design of the enhanced coolant purification systems for the European HCLL and HCPB test blanket modules

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    The Coolant Purification Systems (CPSs) is one of the most relevant ancillary systems of European Helium Cooled Lead Lithium (HCLL) and Helium Cooled Pebble Bed (HCPB) Test Blanket Modules (TBMs) which are currently in the preliminary design phase in view of their installation and operation in ITER. The CPS implements mainly two functions: the extraction and concentration of the tritium permeated from the TBM modules into the primary cooling circuit and the chemistry control of helium primary coolant. During the HCLL and HCPB-TBSs (Test Blanket Systems) Conceptual Design Review (CDR) in 2015 it was recognized the need of reducing the tritium permeation into the Port Cell #16 of ITER. To achieve this and, then, to lower the tritium partial pressure in the Helium Cooling Systems in normal operation, the helium flow-rate treated by each CPS has been increased of almost one order of magnitude. In 2017, to satisfy the CDR outcomes and the new design requirements requested by Fusion for Energy (F4E, the European Domestic Agency for ITER), ENEA performed a preliminary design of the “enhanced” CPSs. This paper presents the current design of the “enhanced” CPSs, focusing on design requirements, assumptions, selection of technologies and preliminary components sizing

    The importance of simulation in team training on obstetric emergencies: results of the first phase of the national plan for continuous medical training

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    As emergências obstétricas são eventos raros e inesperados. O modelo tradicional para a formação médica nestes eventos críticos apresenta limitações óbvias. Dada a variedade de competências técnicas e o ambiente de alto risco em que são aplicadas, a obstetrícia é uma especialidade que se adequa totalmente ao treino através de técnicas ou tecnologias aplicadas à Medicina ou Obstetrícia. Este modelo de aprendizagem constitui uma oportunidade para aprender e treinar competências técnicas simples e complexas, bem como a oportunidade para ensaiar e aprender com os erros, sem riscos para os doentes. Assim, elaborámos um estudo com o objectivo de avaliar a percepção dos formandos relativamente a quais os factores associados à falibilidade humana antes e após a formação clínica com simulação; comparar o nível de confiança para a resolução de situações emergentes obstétricas entre internos e especialistas com mais de 5 anos de experiência, antes e após uma acção formativa em gestão de eventos críticos de Obstetrícia; determinar o valor atribuído pelos formandos à simulação como instrumento formativo em cuidados emergentes. Nesta fase do programa, correspondente às 3 primeiras sessões formativas em gestão de eventos críticos de Obstetrícia, participaram 31 médicos. Após a acção formativa, verificaram-se alterações na percepção dos formandos relativamente a quais os factores associados à falibilidade humana, no nível de confiança para a resolução de situações emergentes obstétricas e no valor atribuído pelos formandos à simulação como recurso pedagógico

    Idiopathic Eruptive Macular Pigmentation in Childhood

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    A pigmentação macular eruptiva idiopática é uma patologia rara, que se caracteriza pelo aparecimento de múltiplas máculas de cor acastanhada, assintomáticas, que envolvem mais frequentemente a região cervical, o tronco e a região proximal dos membros. A incidência parece ser maior durante a infância e adolescência. Trata-se de uma entidade distinta do eritema discrómico perstans, de natureza benigna e, observando-se habitualmente remissão espontânea ao fim de alguns meses a anos, sem necessidade de tratamento ou investigação clínica adicional

    Fígado gordo causado por glicogenose tipo IX: Uma pequena série de casos em crianças

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    Background: The prevalence of non-alcoholic fatty liver disease (NAFLD) affecting children and adolescents has increased dramatically in recent years. This increase is most probably related to the obesity pandemic and the high consumption of fructose. However, hepatic steatosis has some rare causes (e.g., some metabolic diseases) of which clinicians should be aware, particularly (but not only) when patients are non-obese or non-overweight. Differential diagnosis is notably important when pathologies have a specific treatment, such as for glycogenosis type IX (GSD-IX). Aims: To contribute to the knowledge on the differential diagnosis of NAFLD in paediatric age and to the clinical, biochemical, molecular, and histological characterisations of GSD-IX, a rare metabolic disorder. Methods: We performed a retrospective study of a small series of cases (n = 3) of GSD-IX diagnosed in the past 6 years, who were currently being followed up in the Units of Gastroenterology or Metabolic Diseases of the Paediatric Division of our hospital and whose clinical presentation was NAFLD in paediatric age. Results: Three male patients were diagnosed with NAFLD before 2 years of age, 2 with confirmed diagnosis before the age of 3 years (alanine aminotransferase [ALT], liver ultrasound, and molecular analysis) and 1 whose diagnosis was confirmed at 11 years (ALT, liver ultrasound, liver histology, and molecular analysis). None of the patients were obese or overweight, and the daily fructose consumption was unknown. The outcome was favourable in all 3 patients, with follow-up periods ranging from 2 to 6 years. Conclusion: The decision on how far the search for secondary causes of NAFLD should go can be difficult, and GSD-IX must be on the list of possible causes.Introdução: A prevalência de fígado gordo não-alcoólico (NAFLD) afetando crianças e adolescentes, aumentou enormemente nos últimos anos. Este aumento está muito provavelmente relacionado com a pandemia de obesidade e o elevado consumo de fructose. No entanto, a esteatose hepática pode ser causada por algumas entidades raras (por exemplo, doenças metabólicas) para as quais os clínicos devem estar alertados, particularmente (mas não só) quando os doentes não são obesos nem têm excesso de peso. O diagnóstico diferencial é especialmente importante quando as patologias têm um tratamento específico, como é o caso da Glicogenose tipo IX (GSD-IX). Objetivos: Contribuir para o conhecimento dos diagnósticos diferenciais de NAFLD em idade pediátrica, e contribuir para a caracterização clínica, bioquímica, molecular e histológica da GSD-IX, uma doença metabólica rara. Metodologia:Estudo retrospectivo de uma pequena série de casos (n = 3) de GSD-IX diagnosticados nos últimos 6 anos, atualmente em seguimento nas Unidades de Gastrenterologia ou de Doenças Metabólicas do Serviço de Pediatria do nosso hospital, e cuja forma de apresentação clínica foi NALFLD em idade pediátrica. Resultados: Três doentes de sexo masculino foram diagnosticados com NAFLD antes dos 2 anos de idade, dois com diagnóstico confirmado antes dos 3 anos (alanina aminotransferase [ALT], ecografia hepática, e estudo molecular), e um cujo diagnóstico foi confirmado aos 11 anos (ALT, ecografia e histologia hepáticas, e estudo molecular). Nenhum dos doentes era obeso ou tinha excesso de peso, e o consumo diário de frutose era desconhecido. A evolução foi favorável nos três doentes, com um tempo de seguimento que variouentre 2 e 6 anos. Conclusões: A decisão sobre até onde a pesquisa de causas secundárias de NAFLD deve ir pode ser difícil, e a GSD-IX deve estar na lista de causas possíveis.info:eu-repo/semantics/publishedVersio

    Variable stars in the Fornax dSph Galaxy. I. The Globular Cluster Fornax 4

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    Variable stars have been identified for the first time in Fornax 4, the globular cluster located near the center of the Fornax dwarf spheroidal galaxy. By applying the image subtraction technique to B,V time series photometry obtained with the MagIC camera of the 6.5-m Magellan/Clay telescope and with the wide field imager of the 4-m Blanco/CTIO telescope, we detected 27 RR Lyrae stars (22 fundamental mode, 3 first overtone, and 2 double-mode pulsators) in a 2.4'x2.4' area centered on Fornax 4. The average and minimum periods of the ab-type RR Lyrae stars, = 0.594 d and P(ab,min)=0.5191 d, respectively, as well as the revised position of the cluster in the horizontal branch type--metallicity plane, all consistently point to an Oosterhoff-intermediate status for the cluster, unlike what is seen for the vast majority of Galactic globular clusters, but in agreement with previous indications for the other globular clusters in Fornax. The average apparent magnitude of the RR Lyrae stars located within 30 arcsec from the cluster center is =21.43 +/- 0.03 mag (sigma=0.10 mag, average on 12 stars), leading to a true distance modulus of (m-M)o=20.64 +/- 0.09 mag or (m-M)o=20.53 +/- 0.09 mag, depending on whether a low ([Fe/H]=-2.0) or a moderately high ([Fe/H]=-1.5) metallicity is adopted.Comment: Apj, in pres

    Variable stars in the Fornax dSph Galaxy. II. Pulsating stars below the horizontal branch

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    We have carried out an intensive survey of the northern region of the Fornax dwarf spheroidal galaxy with the aim of detecting the galaxy's short--period pulsating stars (P<0.25 days). Observations collected over three consecutive nights with the Wide Field Imager of the 2.2m MPI telescope at ESO allowed us to detect 85 high-amplitude (0.20-1.00 mag in B-light) variable stars with periods in the range from 0.046 to 0.126 days, similar to SX Phoenicis stars in Galactic metal-poor stellar populations. The plots of the observed periods vs. the B and V magnitudes show a dispersion largely exceeding the observational errors. To disentangle the matter, we separated the first-overtone from the fundamental-mode pulsators and tentatively identified a group of subluminous variables, about 0.35 mag fainter than the others. Their nature as either metal-poor intermediate-age stars or stars formed by the merging of close binary systems is discussed. The rich sample of the Fornax variables also led us to reconstruct the Period-Luminosity relation for short-period pulsating stars. An excellent linear fit, M(V)=-1.83(+/-0.08)-3.65(+/-0.07) log P(fund), was obtained using 153 Delta Scuti and SX Phoenicis stars in a number of different stellar systems.Comment: 11 pages plus 1 on-line figure and 1 on-line table; accepted for publication in ApJ. Part of this work has been the subject of the Laurea thesis of LDA. His supervisor and our colleague, Prof. Laura E. Pasinetti, suddendly passed away on September 13, 2006. Several astronomers have been trained under her tutelage and we gratefully honor her memor

    No evidence that wild red deer (Cervus elaphus) on the Iberian Peninsula are a reservoir of Mycobacterium avium subspecies paratuberculosis infection

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    The potential role of red deer (Cervus elaphus) as a reservoir of Mycobacterium avium subspecies paratuberculosis (MAP) infection is largely unknown. A total of 332 wild red deer were investigated using post-mortem examination, bacteriology and serology. Only three animals (1.12%) were found to have lesions on histopathological examination and no MAP bacteria were recovered on culture. The results suggest it is unlikely that wild red deer make a significant contribution to the maintenance of MAP infection in the region. The cross-reactivity of the ELISAs used indicates this diagnostic modality is ineffective in the detection of MAP infection in this species. The implications of these results for the control of this important pathogen in both livestock and wildlife are discussed
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