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This research aims to present a study on the constitution of the profiles of new Portuguese Language teachers in São Luís – MA, through discursive analysis of Supervised Internship Reports in High School. Therefore, the Critical Discourse Analysis proposed by Chouliaraki and Fairclough (1999), Fairclough (2001, 2003), who understand the importance of the transdisciplinary relationship between Linguistics and various areas of scientific knowledge, is used as a theoretical-methodological foundation. The investigation corpus is composed of six reports, with two fragments of each one of them. For the critical-discursive analysis, in this work, the representational meaning (discourse as a mode of representation) and the analytical category interdiscursivity that should seek to identify the types of discourse and how they are articulated were highlighted. The results point to identity representations built through socio-discursive practices, subjects and disciplinary institutions that design, at a certain level, what is, and how language teaching is done., Esta pesquisa objetiva apresentar um estudo sobre a constituição dos perfis de novos/as professores/as de Língua Portuguesa em São Luís – MA, através de análises discursivas de Relatórios de Estágio Supervisionado do Ensino Médio. Para tanto, utiliza-se como alicerce teórico-metodológico a Análise de Discurso Crítica proposta por Chouliaraki e Fairclough (1999), Fairclough (2001, 2003), que entendem a importância da relação transdisciplinar entre a Linguística e diversas áreas do conhecimento científico. O corpus de investigação é composto por seis relatórios, produzidos no ano de 2019. Para as análises crítico-discursivas, neste trabalho, destacaram-se o significado representacional (discurso como modo de representação) e a categoria analítica interdiscursividade que buscou identificar os tipos de discurso e como eles se articulam. Os resultados apontam para representações identitárias construídas mediante práticas sociodiscursivas, sujeitos e instituições disciplinadoras que desenham, em certo nível, o que é e como se faz a docência no ensino de língua.

BackgroundMucopolysaccharidosis type I (MPS I) results in significant disease burden and early treatment is important for optimal outcomes. Recognition of short stature and growth failure as symptoms of MPS I among pediatric endocrinologists may lead to earlier diagnosis and treatment.Case presentationA male patient first began experiencing hip pain at 5 years of age and was referred to an endocrinologist for short stature at age 7. Clinical history included recurrent respiratory infections, sleep apnea, moderate joint contractures, mild facial dysmorphic features, scoliosis, and umbilical hernia. Height was more than - 2 SD below the median at all time points. Growth velocity was below the 3rd percentile. Treatment for short stature included leuprolide acetate and recombinant human growth hormone. The patient was diagnosed with MPS I and began enzyme replacement therapy with laronidase at age 18.ConclusionsThe case study patient had many symptoms of MPS I yet remained undiagnosed for 11 years after presenting with short stature. The appropriate path to MPS I diagnosis when patients present with short stature and/or growth failure plus one or more of the common signs of attenuated disease is described. Improved awareness regarding association of short stature and growth failure with attenuated MPS I is needed since early identification and treatment significantly decreases disease burden.

Background: Fabry disease is an inherited disorder of glycolipid metabolism with progressive involvement of multiple organs, including the gastrointestinal tract, in classically affected male patients. Clinical presentations in males with later-onset Fabry phenotypes are more heterogeneous and largely dependent on the level of residual α-galactosidase A activity. Methods: We assessed agalsidase beta treatment outcomes of gastrointestinal symptoms in adult males with classic or later-onset Fabry disease. Self-reports of abdominal pain and diarrhea (‘present’/’not present’ since previous assessment) at last clinical visit (≥0.5 year of follow-up) were compared with treatment-baseline. Results: Classic male patients were considerably younger at first treatment than the fewer males with later-onset phenotypes (36 vs. ~47 years) and reported gastrointestinal symptoms more frequently at baseline (abdominal pain: 56% vs. 13%; diarrhea: 57% vs. 23%). As compared with baseline, significantly fewer classic patients reported abdominal pain after a median of 4.7 years of treatment (N = 171, 56% vs. 41%, P < 0.001). Moreover, significantly fewer patients reported diarrhea after 5.5 years of follow-up (N = 169, 57% vs. 47%, P < 0.05). Among the males with later-onset phenotypes, albeit statistically non-significant, abdominal pain reports reduced after a median of 4.2 years (N = 48, 13% vs. 4%) and diarrhea reports reduced after a median of 4.4 years of treatment (N = 47, 23% vs. 13%). Conclusions: Sustained treatment with agalsidase beta was associated with improvement in abdominal pain and diarrhea in a significant proportion of classic male Fabry patients. Males with later-onset phenotypes reported gastrointestinal symptoms much less frequently at baseline as compared with classic patients, and non-significant reductions were observed.

Aims: Long-term treatment effect studies in large female Fabry patient groups are challenging to design because of phenotype heterogeneity and lack of appropriate comparison groups, and have not been reported. We compared long-term cardiomyopathy and kidney function outcomes after agalsidase beta treatment with preceding treatment-naive outcomes. Methods and results: Self-controlled pretreatment and post-treatment comparison (piecewise mixed linear modelling) included Fabry female patients ≥18 years at treatment initiation who received agalsidase beta (0.9–1.1 mg/kg every other week) for ≥2 years, with ≥2 pretreatment and ≥2 post-treatment outcome measurements during 10-year follow-up. Left ventricular posterior wall thickness (LVPWT)/interventricular septal thickness (IVST) and estimated glomerular filtration rate (eGFR, Chronic Kidney Disease Epidemiology Collaboration creatinine equation) analyses included 42 and 86 patients, respectively, aged 50.0 and 46.3 years at treatment initiation, respectively. LVPWT and IVST increased pretreatment (follow-up 3.5 years) but stabilized during 3.6 years of treatment (LVPWT: n = 38, slope difference [95% confidence interval (CI)] = −0.41 [−0.68, −0.15] mm/year, Ppre–post difference <0.01; IVST: n = 38, slope difference = −0.32 [−0.67, 0.02] mm/year, Ppre–post difference = 0.07). These findings were not modified by renal involvement or antiproteinuric agent use. Compared with the treatment-naive period (follow-up 3.6 years), eGFR decline remained modest and stabilized within normal ranges during 4.1 years of treatment (slope difference, 95% CI: −0.13 [−1.15, 0.89] mL/min/1.73m2/year, Ppre–post difference = 0.80). Conclusions: Cardiac hypertrophy, progressing during pretreatment follow-up, appeared to stabilize during sustained agalsidase beta treatment. eGFR decline remained within normal ranges. This suggests that treatment may prevent further Fabry-related progression of car

Aims: Long-term treatment effect studies in large female Fabry patient groups are challenging to design because of phenotype heterogeneity and lack of appropriate comparison groups, and have not been reported. We compared long-term cardiomyopathy and kidney function outcomes after agalsidase beta treatment with preceding treatment-naive outcomes. Methods and results: Self-controlled pretreatment and post-treatment comparison (piecewise mixed linear modelling) included Fabry female patients ≥18 years at treatment initiation who received agalsidase beta (0.9-1.1 mg/kg every other week) for ≥2 years, with ≥2 pretreatment and ≥2 post-treatment outcome measurements during 10-year follow-up. Left ventricular posterior wall thickness (LVPWT)/interventricular septal thickness (IVST) and estimated glomerular filtration rate (eGFR, Chronic Kidney Disease Epidemiology Collaboration creatinine equation) analyses included 42 and 86 patients, respectively, aged 50.0 and 46.3 years at treatment initiation, respectively. LVPWT and IVST increased pretreatment (follow-up 3.5 years) but stabilized during 3.6 years of treatment (LVPWT: n = 38, slope difference [95% confidence interval (CI)] = -0.41 [-0.68, -0.15] mm/year, Ppre-post difference <0.01; IVST: n = 38, slope difference = -0.32 [-0.67, 0.02] mm/year, Ppre-post difference = 0.07). These findings were not modified by renal involvement or antiproteinuric agent use. Compared with the treatment-naive period (follow-up 3.6 years), eGFR decline remained modest and stabilized within normal ranges during 4.1 years of treatment (slope difference, 95% CI: -0.13 [-1.15, 0.89] mL/min/1.73m2 /year, Ppre-post difference = 0.80). Conclusions: Cardiac hypertrophy, progressing during pretreatment follow-up, appeared to stabilize during sustained agalsidase beta treatment. eGFR decline remained within normal ranges. This suggests that treatment may prevent further Fabry-related progression of cardiomyopathy in female patients and maintain norma

Plastics and other artificial materials pose new risks to the health of the ocean. Anthropogenic debris travels across large distances and is ubiquitous in the water and on shorelines, yet, observations of its sources, composition, pathways, and distributions in the ocean are very sparse and inaccurate. Total amounts of plastics and other man-made debris in the ocean and on the shore, temporal trends in these amounts under exponentially increasing production, as well as degradation processes, vertical fluxes, and time scales are largely unknown. Present ocean circulation models are not able to accurately simulate drift of debris because of its complex hydrodynamics. In this paper we discuss the structure of the future integrated marine debris observing system (IMDOS) that is required to provide long-term monitoring of the state of this anthropogenic pollution and support operational activities to mitigate impacts on the ecosystem and on the safety of maritime activity. The proposed observing system integrates remote sensing and in situ observations. Also, models are used to optimize the design of the system and, in turn, they will be gradually improved using the products of the system. Remote sensing technologies will provide spatially coherent coverage and consistent surveying time series at local to global scale. Optical sensors, including high-resolution imaging, multi- and hyperspectral, fluorescence, and Raman technologies, as well as SAR will be used to measure different types of debris. They will be implemented in a variety of platforms, from hand-held tools to ship-, buoy-, aircraft-, and satellite-based sensors. A network of in situ observations, including reports from volunteers, citizen scientists and ships of opportunity, will be developed to provide data for calibration/validation of remote sensors and to monitor the spread of plastic pollution and other marine debris. IMDOS will interact with other observing systems monitoring physical, che

So wie das Ende des Brexit-Prozesses weiterhin nicht absehbar bleibt, sind die Folgen des Austritts des UK aus der EU – mit oder ohne Abkommen – kaum abzumessen. Dieser Band versucht eine Zwischenbilanz, von Grundfragen zur Souveränität, um deren Wiedergewinnung es den Brexitern zu gehen scheint, über Modelle differenzierter Integration und den Grundrechtsschutz bis hin zur Demokratie, die in mancherlei Weise auf die Probe gestellt wird. Wie ist der Binnenmarkt betroffen, wie die vom EuGH vielleicht zu weitgehend etablierten sozialen Rechte, wie die Kontrolle der Einwanderung in das UK? – Dem ist Teil II des Sammelbandes gewidmet. Der letzte Teil betrifft mit der Währungs- und Finanzpolitik sowie der Gemeinsamen Außen- und Sicherheitspolitik Bereiche, die nur begrenzt der supranationalen Disziplin unterliegen und wo das UK gleichwohl eine wichtige Rolle spielt und auch künftig spielen könnte. Vieles stellt sich heute anders dar, als man vor dem Referendum von 2016 erwartet hätte.

Este volume de quase 1600 páginas é constituído por 60 estudos linguísticos e filológicos oferecidos a Ivo Castro por colegas de Portugal, Brasil, Espanha, Itália, Reino Unido, Suiça, Alemanha, Holanda e Polónia. Foram convidados a contribuir para esta homenagem todos os participantes, com comunicação, no IV Congresso Internacional de Linguística Histórica (IVo CILH), dedicado a Ivo Castro, que se realizou em Lisboa, de 17 a 21 de julho de 2017, com a presença de mais de duas centenas de colegas, estudantes, discípulos e amigos (cf. http://ivo-cilh.letras.ulisboa.pt/). As áreas temáticas correspondentes às sete secções do IVº CILH estão representadas neste volume, mas de forma díspar, o que nos fez optar por não organizar o índice em secções temáticas (as do congresso foram: crítica textual, edição de texto e humanidades digitais; mudança linguística e linguística teórica; léxico e etimologia; sociolinguística e dialetologia; história social, história da escrita, tradições discursivas e periodização; historiografia linguística, pedagogia e tradução; música, cultura e literatura). Se na organização do congresso as secções temáticas ajudavam os participantes a orientar-se dentro de um programa longo, com sessões paralelas e, portanto, sem a opção de se ouvir tudo, o volume publicado permite tanto a leitura parcelar como integral, mas à discrição do leitor, viajando pelos diferentes textos ao sabor dos seus interesses, objetivos e itinerários.

Apesar dos avanços alcançados com a adoção de programas de segurança e controle de alimentos, os sistemas de vigilância ainda relatam a ocorrência de doenças transmitidas por alimentos (DTAs) em países desenvolvidos e em desenvolvimento, e a carne de peru é frequentemente envolvida em surtos, atingindo várias pessoas anualmente. As bactérias são responsáveis por mais de 66% dos casos de DTAs que resultam em hospitalizações, destacando-se principalmente Salmonella spp., Campylobacter sp., Escherichia coli, Listeria monocytogenes, Staphylococcus spp., Clostridium botulinum e Clostridium perfringens. As fontes de contaminação de carcaças de peru são semelhantes às de frango, entretanto, os relatos sobre o envolvimento da carne de peru em DTAs e seu risco a saúde pública são escassos. Assim, esta revisão se concentrou em identificar os microorganismos relatados nos surtos de carne de peru e seus fatores de risco e nas principais fontes de contaminação, bem como as limitações no estudo de sua epidemiologia desde a produção até os consumidores. Um alto risco foi atribuído ao gênero Salmonella spp., Escherichia coli, Staphylococcus spp. e Campylobacter sp., sendo o último, considerado negligenciado nos países em desenvolvimento. Este estudo mostrou a necessidade de um maior incentivo às pesquisas sobre as condições sanitárias da carne de peru processada no Brasil, bem como a necessidade de monitorar esta carne vendida no país.

Issues related to animal welfare and the humane slaughter of animals are gaining importance in the scientific community and among consumers, by the close relationship with the final quality of meat, as well to avoid unnecessary suffering on the steps leading up to slaughter. The objective of this study was to evaluate the effectiveness of stunning in cattle slaughterhouse located in the State of São Paulo. The animals were stunned by penetrating captive bolt and then observed the number of shots applied to each animal, and the presence of signs of sensitivity of animals (eye movements, rhythmic breathing, vocalization and attempts to correct posture) on bleeding. Of 200 animals evaluated, 140 (70%) showed signs of unconsciousness at the first shooting, 39 (19.5%) in the second and in 21 (10.5%) took three or more shots. The signals observed in 14 (7%) animals showed attempt to correct posture and 186 (93%) showed no signs of sensitivity. The results were not satisfactory, evidencing the need to train staff responsible for the stunning stage, as well the need to use the stun box with head restraint and a real monitoring that ensures animal welfare during the time preceding slaughter, Questões relacionadas ao bem-estar animal e ao abate humanitário de animais de açougue vêm ganhando cada vez mais importância no meio científico e entre os consumidores, seja pela estreita relação com a qualidade final da carne, ou também para se evitar o sofrimento desnecessário nas etapas que antecedem o abate dos mesmos. O objetivo do trabalho foi avaliar a eficiência da insensibilização em bovinos em matadouro-frigorífico localizado na Região Noroeste do Estado de São Paulo. Os animais foram submetidos a insensibilização por pistola pneumática de penetração e, então, observado o número de disparos aplicado em cada animal, além dos sinais de sensibilidade dos animais (movimento ocular, respiração rítmica, vocalização e tentativa de correção de postura) na calha de sangria. De 200 animais avaliados, 140 (70%) demonstraram sinais de inconsciência logo no primeiro disparo, 39 (19,5%) no segundo disparo e em 21 (10,5%) foram necessários três ou mais disparos. Dos sinais observados, 14 (7%) animais apresentaram tentativa de correção de postura e 186 (93%) não apresentaram sinais de sensibilidade. Os resultados não foram satisfatórios, permitindo evidenciar a necessidade de treinamento de funcionários encarregados pela etapa de insensibilização, assim como a necessidade de utilização de box de atordoamento com contenção de cabeça e um real monitoramento que garanta o bem-estar animal durante o momento que antecede o abate.

The aim of this study was to identify suggestives lesions of tuberculosis in animals in slaughterhouses, classify them as calcified or caseous and identify the most affected organs and the brazilian State with the largest number of animals with lesions . Were inspected 38,172 crossbred cattle, males, slaughtered at two slaughterhouses with the Federal Inspection Service - SIF in the northeast of São Paulo. In post-mortem, tuberculous lesions were identified and classified as calcified or caseous. The experimental period was 90 days. Macroscopic lesions were identified, annotated and recorded with the aid of camera. Besides the classification of lesions in calcified or caseous also identified the most affected organ and State of origin of the animals with lesions suggestive of tuberculosis. The organs examined were the lungs, liver, tongue, lymph nodes of head and mediastinal chain. The animals studied showed an incidence of 0.16% of lesions indicative of caseous tuberculosis and 0.11% of lesions indicative of calcified tuberculosis, of which 99.73% had no lesions characteristic of tuberculosis. The most affected organ was the lung and the State of origin with the highest incidence of lesions was São Paulo., Objetivou-se com este estudo identificar lesões sugestivas de tuberculose em bovinos abatidos em matadouros frigoríficos, classificá-las como calcificada ou caseosa e identificar quais os órgãos mais acometidos e o Estado brasileiro com maior número de animais com lesões. Foram inspecionados 38.172 bovinos mestiços, machos, abatidos em dois frigoríficos com Serviço de Inspeção Federal - SIF na região nordeste do Estado de São Paulo. No exame post-mortem foram identificadas as lesões tuberculosas e estas classificadas em calcificada ou caseosa. O período experimental foi de 90 dias. As lesões macroscópicas eram identificadas, anotadas e registradas com auxílio de máquina fotográfica. Além da classificação das lesões em calcificada ou caseosa, também foram identificados os órgãos mais acometidos e o Estado de origem dos animais que apresentaram lesões sugestivas de tuberculose. Os órgãos inspecionados foram os pulmões, fígado, língua, linfonodos da cabeça e linfonodos da cadeia mediastínica. Os animais estudados apresentaram incidência de 0,16% de lesões sugestivas de tuberculose caseosa e 0,11% de lesões sugestivas de tuberculose calcificada, sendo que 99,73% não apresentaram lesões características de tuberculose. O órgão mais acometido foi o pulmão e o Estado de origem com maior incidência de animais com lesões foi o de São Paulo.

Taking into account the importance of meats and its products on population diet, the high consumption of these, as the importance of food handlers training in these establishments and the necessity to adopt good handling practices, the aim of this study was to implant and also check of good handling practices programs on meat marketplaces, important link in the production chain of meat with consumers, in São José do Rio Preto city, São Paulo State. Overall, several features were in disagreement with the current law, since the building situation until the manner that workers handle with meat products. Thus, a rigorous and constant inspection must be done on meat marketplaces, as qualified workers requirement to handle and properly conserve the products in order to keep the food quality and safety, reducing the risks to those who consume them and thus providing a nutritious and sustainable food for the population, Considerando-se a importância dos diferentes tipos de carnes e seus derivados na alimentação da população, no consumo elevado dessas, na importância da capacitação dos manipuladores de alimentos e na necessidade de implantação das boas práticas de manipulação nestes estabelecimentos, o presente estudo objetivou implantar e avaliar o programa de boas práticas de manipulação em açougues, elo importante da cadeia produtiva de carne com o consumidor, do Município de São José do Rio Preto/SP. De forma geral, vários aspectos observados estavam em desacordo com a legislação vigente, desde situação da edificação até a manipulação dos produtos cárneos. Assim, é necessária uma rigorosa e constante fiscalização nos estabelecimentos que comercializam produtos cárneos, com adoção de medidas mais drásticas e com a exigência da presença de profissionais qualificados para a correta manipulação e conservação dos produtos, para a manutenção da qualidade final e a segurança dos alimentos, minimizando os riscos para os consumidores.

In order to complement the histopathological study of juveniles and tadpoles of the bullfrog, Lithobathes catesbeianus, fed commercial diet used by frog farms in Rio de Janeiro, containing 40% crude protein, we performed necropsy and histopathology of the liver to establish a relationship between the quality of crude protein in the diet and animal health. For this, it was used twenty breeding male bullfrogs, with average weight 591.30 g (± 91.90 g) and length 165.02 mm (± 14.22 mm), and ten females with average weight and length of 629.80 g (± 134.47 g) and 169.32 mm (± 21.82 mm). The liver histopathology showed hyperemia, high number of melanomacrophages and cytoplasmic rarefaction, probably due to protein deficiency and fatty liver degeneration and presence of inflammatory processes. These lesions indicate a degenerative nutritional process. These findings suggest that the animals were fed with proteins of low biological value, indicating poor quality of feed, undermining the sanity. The impairment of liver function by these injuries will lead to reduced availability of precursors of sex hormones, since the liver is important in the metabolism of the same, and reproductive performance of these animals may be impaired.

To evaluate the effect of agalsidase beta on longitudinal health-related quality of life in patients with Fabry disease.

[Resumen] Stella Matutina es la segunda fase del proyecto del arquitecto John Pawson para el Monasterio de Nuestra Señora de Novy Dvur (República Checa). En él se reúnen los principios, fundamentos y bases del ideal cisterciense pero a través de detalles contemporáneos, que aún se conectan con las preocupaciones de San Bernardo en su «Apología al Abad Guillerme» (siglo XII). Se ha conectado la espiritualidad cisterciense a la arquitectura contemporánea por los principios cistercienses, por el minimalismo contemporáneo o por su impacto, ya sea en la región dónde se inserta, ya sea como marco histórico arquitectónico del siglo XXI, siendo además una forma de catequesis y evangelización., [Abstract] Stella Matutina is the 2nd phase of John Pawson’s project for the Monastery of Our Lady of Novy Dvur (Czech Republic). The principles, fundamentals and basis of the Cistercian ideal are met, through contemporary details, which are still connected with the thoughts of Saint Bernard in his «Apology to Abbot William» (12th century). The Cistercian Spirituality was connected to Contemporary Architecture through Cistercian principles, contemporary minimalist principles or even for its impact, in the region where it is inserted or as a 21st century historical and architectonic landmark, thus being also a way catechizing and evangelization.

[Resumen] En esta comunicación se presentará los principios, fundamentos y bases del ideal cisterciense con su materialización arquitectónica, de gran simplicidad y ascetismo, tanto en su génesis en el siglo XII como ahora en el arranque del nuevo milenio. Se entiende un monasterio cisterciense como un lugar lleno de ideales y de espiritualidad, dónde se busca a Dios. El monasterio cisterciense es así un ideal que tiene una representación y materialización que, con los siglos transcurridos, sufrieron algunas transformaciones y adaptaciones. Será el objetivo de este trabajo hacer un paralelo entre la austera arquitectura del Císter del siglo XII, del plan ad quadratum de las iglesias y de toda la influencia del aparente plan-tipo de sus monasterios, en la arquitectura de los siglos siguientes. Se hará una conexión entre todos estos principios y la reciente rehabilitación de una antigua granja en la actual Republica Checa. El arquitecto fue el minimalista John Pawson y la granja es actualmente el Monasterio de Novy Dvur, el primer monasterio cisterciense del post-comunismo en la Republica Checa. Hay cuestiones para las cuales se buscará respuesta, ¿Cuáles fueran los ideales arquitectónicos cistercienses y como se materializaron a lo largo de los siglos?, ¿Como se entendió, de modo arquitectónico, la espiritualidad cisterciense desde la Apología de San Bernardo hasta nuestros días?, ¿Cuáles fueron los ideales y la realidad cistercienses en su génesis del siglo XII y cual fue su legado?, ¿Y en nuestros días, como se materializan los ideales cistercienses? O sea, ¿Cómo es posible que esta espiritualidad tenga una materialización arquitectónica y que siga influenciando arquitectos contemporáneos, no solo en su obra como también instigándoles a la creación de una arquitectura llena de espiritualidad como es el caso del Monasterio de Novy Dvur? Esto es, del Císter del siglo XII hasta el minimum del siglo XXI.