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Behçet's disease is a chronic relapsing multisystem autoinflammatory condition, in which cardiac involvement is rare, but among the most life-threatening complications. Treatment is largely empirical, and is aimed at suppressing vasculitis. In this role glucocorticoids and colchicine are frequently used.We present the case of a 42-year-old male with previously diagnosed Behçet's disease presenting to our emergency department with an anterior-inferior STEMI. He presented combined thrombosis of the distal anterior descending coronary artery and proximal right coronary artery, and was treated with sequential primary percutaneous coronary interventions and implantation of drug-eluting stents, but required two interventions due to high thrombotic load.His clinical course during hospitalization was good, with no systolic dysfunction at discharge. During follow-up, he has so far had no new cardiovascular events. Resumo: A doença de Behçet (BD) é uma doença autoimune crónica e recidivante, na qual o envolvimento cardíaco é muito raro, mas encontra-se entre as complicações fatais. O tratamento é em grande parte empírico e destina-se a suprimir a vasculite. Nesse papel, os glicocorticoides e a colchicina são frequentemente utilizados.Apresentamos o caso de um homem de 42 anos, com diagnóstico prévio de doença de Behçet que recorreu ao nosso serviço de emergência com STEMI anteroinferior. Apresentou trombose simultânea da artéria coronária descendente anterior distal e da artéria coronária direita proximal e foi tratada com intervenção coronária percutânea primária com stent revestido de drogas de forma sequencial, mas necessitando de duas intervenções devido à recorrência trombótica.Teve boa evolução clínica durante o internamento, não apresentando disfunção sistólica na alta. Durante o seguimento, até à data, não ocorreu novo evento cardiovascular.

Abstract The production of açaí seed waste from the commercial and extractive exploitation of the Euterpe oleraceae palm tree is a serious problem that contributes to environmental contamination and production of greenhouse gases, a fact that suggests the need for an environmentally correct destination for this waste produced on a large scale. To this end, this study was conducted to evaluate the potential of acaí seed biochar (BCA) in mitigating the toxic effects of copper in Brazilian mahogany plants, analyzing biometrics and gas exchange. The experimental design was in randomized blocks, with five blocks, in a 4 × 3 factorial scheme, corresponding to the control (without Cu) and three concentration of Cu (200, 400, and 600 mg Cu kg−1) and three levels of BCA (0%, 5% and 10%) proportional to the amount of soil in the pots, totaling sixty experimental units. The use of 5% BCA in soils contaminated with up to 200 mg kg−1 Cu promoted biometric increase (height, diameter, number of leaves), maintaining gas exchange (photosynthesis, stomatal conductance, transpiration, internal carbon and internal/external carbon), and consequently, maintaining water use efficiency in plants under abiotic stress, resulting in plant growth. The findings of this study allow us to indicate the use of biochar in remediating and improving the growth of plants grown in copper-contaminated soils. The production of biochar from açaí seeds is an ecologically sustainable alternative, because it reduces its accumulation on public roads and contributes to reducing soil pollution. In the context of public policies, biochar production could be a source of income in the context of the bioeconomy and circular economy practiced in the Amazon, because it is produced in large quantities.

Resumo: Os autores apresentam um raro caso de miocardiopatia hipertrófica (MCH) associada a ventrículo esquerdo não compactado (VENC) e fístulas das artérias coronárias para o ventrículo esquerdo (VE) numa doente de 42 anos, que se apresenta com enfarte agudo do miocárdio sem elevação do segmento ST (EAMSEST). A coronariografia, a ecocardiografia transtorácica (ETT) e ressonância magnética cardíaca (RMC) revelaram as alterações estruturais do VE e da árvore coronária. Abstract: The authors present a rare case of hypertrophic cardiomyopathy associated with left ventricular noncompaction cardiomyopathy and coronary artery-left ventricular fistulae in a 42-year-old woman presenting with non-ST-elevation myocardial infarction. Coronary angiography, transthoracic echocardiography and cardiac magnetic resonance revealed the structural abnormalities of the left ventricle and the coronary tree. Palavras-chave: Miocardiopatia hipertrófica, Ventrículo esquerdo não compactado, Fístula coronário-ventricular, Enfarte do miocárdio embólico, Recesso intramiocárdico, Mutação genética, Keywords: Hypertrophic cardiomyopathy, Left ventricular noncompaction cardiomyopathy, Coronary artery-left ventricular fistulae, Embolic myocardial infarction, Intramyocardial recess, Genetic mutation

Resumo: O movimento anterior sistólico (SAM) é uma complicação pós cirúrgica da valvuloplastia mitral, sendo a sua incidência de 5-10%. O reconhecimento precoce dos sinais e sintomas de SAM é imperativo no delinear de estratégia terapêutica nesses pacientes. Este artigo foca os principais mecanismos fisiopatológicos do SAM dinâmico e modalidades de tratamento descritas na literatura. Os autores descrevem um caso clínico e as imagens ecocardiográficas captadas ilustrando a relevância clínica do mecanismo envolvido, na tentativa de esclarecer uma questão suscitada: reintervenção cirúrgica necessária? Abstract: Systolic anterior motion (SAM) is a postoperative complication of mitral valve repair, with an incidence of 5 to 10%. Early recognition of the signs and symptoms of SAM is essential for the management of these patients. This article focuses on the pathophysiology and dynamics of SAM and the treatment strategies described in the literature. The authors present a case study and echocardiographic images illustrating the clinical relevance of the mechanism involved, in order to clarify whether surgical reintervention is necessary. Palavras-chave: Movimento anterior sistólico, Valvuloplastia mitral, Fisiopatologia, Keywords: Systolic anterior motion, Mitral valve repair, Pathophysiology

Resumo: A doença de Fabry é causada por acumulação intracelular de glicoesfingolipidos em vários tecidos, secundária a mutações no gene GLA (Xq22).Classicamente descrita como afectando homens hemizigotos sem actividade residual de alfa-galactosidase-A, sabe-se hoje afectar ambos os sexos com manifestações mais tardias e frustres no feminino. Associa-se a sintomas neurológicos, cutâneos (angioqueratomas), renais, cardiovasculares (hipertrofia ventricular esquerda, alterações do ritmo e espessamento valvular), cocleo-vestibulares e cerebrovasculares.Na ausência de tratamento ocorre dano progressivo de órgãos vitais: doença renal terminal e complicações cardiovasculares e cerebrovasculares potencialmente fatais, implicando redução da qualidade e esperança de vida.Descreve-se o caso de doente do sexo feminino, com antecedentes de acidente vascular cerebral (AVC) isquémico criptogênico com 38 anos e insuficiência renal crónica (IRC) com proteinúria, que se apresenta no serviço de urgência com fibrilhação auricular. O estudo ecocardiográfico revelou hipertrofia concêntrica do ventrículo esquerdo com disfunção diastólica e diminuição do strain longitudinal basal. Englobada num protocolo, de rastreio, foi-lhe diagnosticada doença de Fabry e identificada uma mutação não descrita previamente. Abstract: Fabry disease is caused by intracellular accumulation of glycosphingolipids in various tissues, secondary to mutations in the GLA gene (Xq22).Classically described as affecting hemizygous males with no residual alpha-galactosidase A activity, it is now known to affect both sexes, with later and less severe manifestations in females. The manifestations of this disease are systemic: neurological, cutaneous (angiokeratomas), renal, cardiovascular (left ventricular hypertrophy, valve thickening or rhythm disturbances), cochlear-vestibular, and cerebrovascular. In the absence of treatment there is progressive damage to vital organs with renal failure, stroke, heart failure or rhythm perturbations, leading to severe impairment of quality of life as well as reduced life expectancy.We describe the case of a female patient with a history of cryptogenic ischemic stroke at the age of 38 years and chronic renal failure with proteinuria, who presented to the emergency room with atrial fibrillation. The echocardiogram revealed concentric left ventricular hypertrophy, diastolic dysfunction and decreased longitudinal strain in the basal septum.In the context of a screening protocol, she was diagnosed with Fabry disease and a previously undescribed mutation was identified. Palavras-chave: Doença de Fabry, Miocardiopatias, Hipertrofia ventricular esquerda, 2D Strain, Genética, Keywords: Fabry disease, Cardiomyopathies, Left ventricular hypertrophy, Speckle tracking, Genetics

Miocárdio não compactado (MCNC) é uma cardiopatia congênita rara, resultado da falha na compactação do miocárdio, levando à persistência de trabeculações numerosas e profundas, comunicantes com a cavidade ventricular. Tem como principais manifestações clínicas: insuficiência cardíaca, eventos arrítmicos (supraventriculares ou ventriculares) e episódios de embolismo arterial. Apresenta-se o caso de um cidadão brasileiro residente em Portugal internado por infarto agudo do miocárdio, do qual resultou disfunção sistólica severa do ventrículo esquerdo. No decorrer do estudo diagnosticou-se MCNC. Descreve-se a apresentação clínica, ecocardiográfica (bidimensional e tridimensional), imagem de ressonância magnética nuclear (RMN) e ventriculografia. Discutem-se os critérios diagnósticos e opções terapêuticas.El miocardio no compactado (MCNC) es una cardiopatía congénita rara, resultado de la falla en la compactación del miocardio, y ocasiona la persistencia de trabeculaciones numerosas y profundas, comunicantes con la cavidad ventricular. Tiene como principales manifestaciones clínicas: insuficiencia cardiaca, eventos arrítmicos (supraventriculares o ventriculares) y episodios de embolismo arterial.Left-ventricular non-compaction (LVNC) is a rare congenital cardiopathy, which results from the failure of the myocardial compaction process, leading to the persistence of numerous and deep trabeculations communicating with the ventricular cavity. Its main clinical manifestations are heart failure, arrhythmias (supraventricular or ventricular) and episodes of arterial embolism. The present case reports on a Brazilian patient living in Portugal, who was hospitalized due to acute myocardial infarction, which resulted in severe LV systolic dysfunction. During the patient's assessment, LVNC was diagnosed. The clinical presentation is described, as well as the echocardiographic evaluation (two-dimensional and three-dimensional), nuclear magnetic resonance (NMR) imaging and ventriculography results. The diagnostic criteria and therapeutic options are discussed.

Objetivo: validar uma tecnologia educacional sobre amamentação, no formato de gibi, direcionada à rede de apoio da nutriz. Método: Trata-se de um estudo metodológico para a validação do instrumento elaborado. A análise da validação de conteúdo foi realizada através do Índice de Validade de Conteúdo, e a significância da taxa de concordância adotada foi avaliada a partir do teste binomial. Resultados: Todos os itens ficaram acima da taxa mínima de concordância. No entanto, o p-valor do teste binomial indicou a necessidade de readequação de quatro itens que foram ajustados conforme uma descrição qualitativa dos juízes. As avaliações de cada tópico do formulário apresentaram resultados extremamente satisfatórios, e a menor taxa foi de 0,94. A concordância universal atingiu 0,56, sendo a menor taxa obtida em todo o estudo. Em contrapartida, dos 72 itens avaliados, 41 atingiram a pontuação máxima. A avaliação Global do instrumento foi de 0,96, acima da taxa mínima considerada ideal para este tipo de estudo. Conclusão: O estudo apresentou uma análise elevada na validação da tecnologia educacional sobre amamentação, no formato de gibi, direcionada à rede de apoio da nutriz.

El artículo se centra en la propuesta presentada por Lina Bo Bardi al concurso para la reestructuración urbana del Valle del Anhangabaú, en el centro histórico de São Paulo. El objetivo del concurso, promovido en 1981, era solucionar un problema infraestructural: liberar la zona de un tráfico pesado que no necesitaba entrar en el centro de la ciudad. Descartada por fantasiosa, la propuesta de Lina se oponía por completo a la solución subterránea que ganó el concurso y fue ejecutada. En lugar de un túnel, Lina diseñó un gigantesco viaducto y un parque exuberante. El artículo examina la propuesta de Lina para el Anhangabaú en lo que concierne al rol de la naturaleza en la ciudad, cotejando sus dimensiones programáticas, urbanísticas y figurativas. El trabajo ubica la propuesta en relación con el proceso histórico de reconstrucción del paisaje del valle, a través de registros historiográficos, iconográficos y literarios, subrayando su legado disciplinar y relevancia para el reconocimiento de la cultura arquitectónica y urbanística moderna.

Abstract Background There is considerable burden of illness in hereditary angioedema (HAE). However, instruments to assess health-related quality of life (HRQoL) in HAE are limited. The Angioedema Quality of Life Questionnaire (AE-QoL) was developed to measure HRQoL in patients with recurrent angioedema; the validity of the AE-QoL in patients with HAE is described. Methods To identify disease-related experiences with a focus on the impact of HAE on HRQoL, interviews were conducted with a group of clinician experts and patients with HAE from Canada, France, Germany, Spain, the United Kingdom, and the United States, along with a targeted literature review. Concepts were mapped to the AE-QoL to assess item relevance, interpretation, and conceptual coverage. Cognitive interviews assessed item clarity and relevance. A psychometric validation was performed using data from a phase 3 trial. Results Interviews were conducted with seven clinicians and 40 adult patients. Patients reported 35 unique impacts of HAE on their lives, the most frequent being on work/school, social relationships, physical activities, and emotions, particularly fear/worrying and anxiety. Saturation for these impacts was reached, and all concepts covered in the AE-QoL were reported during the interviews. Patients agreed that the questionnaire items and response options were clear and relevant, and the 4-week recall period was appropriate. The psychometric validation included data from 64 patients. For AE-QoL total scores, excellent internal consistency (Cronbach’s alpha > 0.90), test-retest reliability (intraclass coefficient > 0.80), convergent validity with the Sheehan Disability Scale (r = 0.663), divergent validity with the EQ-5D-5L index (r = 0.292) and EQ-VAS (r = 0.337), and known-groups validity (p

This state-of-the-art review focuses on translanguaging in education. In recent years, scholars have engaged in the conceptualisation of 'translanguaging' (e.g. García, 2009; García & Wei, 2014a; Wei, 2018) as well as in conducting a vast and ever-increasing number of empirical studies, in educational contexts in particular. This article aims to take stock of the different ways in which 'translanguaging' has been conceptualised and of the ways in which it has been interpreted and applied in the study of multilingualism in diverse educational contexts across the globe. Our review exercise shows that 'translanguaging' has been conceptualised within two different approaches, which we propose to call the 'fixed language approach' and the 'fluid languaging approach'. It further shows how 'translanguaging' has been studied within these two approaches in different educational contexts. We finish our review by calling for new methodologies adapted to a linguistics of fluidity and by considering critically translanguaging as a scholarly phenomenon.

This article makes the case for conducting ethnographic research of a multi-scalar nature that links language policy processes and ideologies of language with everyday practices, on the ground, in local schools and classrooms. As with other researchers engaged in the ethnography of language policy (e.g. McCarty, 2011; Johnson, 2013), my concern is with the ways in which language policies in multilingual countries are translated into classroom practice, the ways teachers and school administrators understand and respond to policy changes and the ways in which communication between teachers and learners is shaped by the introduction of a new medium of instruction. The research presented here focuses on language policy and classroom practice in Timor-Leste. On Independence in 2002, Tetum and Portuguese were chosen to be the two official languages of the country and the main languages of teaching and learning in the school system. My main research sites have been primary schools and classrooms in Timor-Leste and I have adopted a linguistic ethnographic approach, combining ethnography with close analysis of classroom discourse and with discourse analysis of policy documents and interviews. I have used the concept of language ideology as an analytical lens in examining the language policy discourses of policymakers and teachers. Teachers in Timor-Leste are regarded by policymakers as the mere facilitators of the process of implementing Tetum and Portuguese language-in-education policy within the education system. Through the analysis of interview data presented here, I show that teachers assumed this role and shared the belief that Tetum and Portuguese were legitimate official languages of Timor-Leste. Then, through analysis of codeswitching in classroom interaction, in one Year 6 classroom, I show how values around Tetum and Portuguese were being discursively constructed by the teacher, particularly in talk around monolingual texts in Portuguese.

Abstract Background: Heart failure (HF) is a highly prevalent syndrome. Although the long-term prognostic factors have been identified in chronic HF, this information is scarcer with respect to patients with acute HF. despite available data in the literature on long-term prognostic factors in chronic HF, data on acute HF patients are more scarce. Objectives: To develop a predictor of unfavorable prognostic events in patients hospitalized for acute HF syndromes, and to characterize a group at higher risk regarding their clinical characteristics, treatment and outcomes. Methods: cohort study of 600 patients admitted for acute HF, defined according to the European Society of Cardiology criteria. Primary endpoint for score derivation was defined as all-cause mortality and / or rehospitalization for HF at 12 months. For score validation, the following endpoints were used: all-cause mortality and / or readmission for HF at 6, 12 and 24 months. The exclusion criteria were: high output HF; patients with acute myocardial infraction, acute myocarditis, infectious endocarditis, pulmonary infection, pulmonary artery hypertension and severe mitral stenosis. Results: 505 patients were included, and prognostic predicting factors at 12 months were identified. One or two points were assigned according to the odds ratio (OR) obtained (p < 0.05). After the total score value was determined, a 4-point cut-off was determined for each ROC curve at 12 months. Two groups were formed according to the number of points, group A < 4 points, and group B = 4 points. Group B was composed of older patients, with higher number of comorbidities and predictors of the combined endpoint at 6, 12 and 24 months, as linearly represented in the survival curves (Log rank). Conclusions: This risk score enabled the identification of a group with worse prognosis at 12 months.

This paper contributes to the building of a sociolinguistics of the south by considering the nature and significance of contemporary language-in-education policy processes in Timor-Leste, and by tracing the ways in which these processes have been shaped by its specific colonial and post-colonial history. In presenting this account, I draw on recent research of a linguistic ethnographic and historical nature conducted in Timor-Leste. The linguistic ethnographic research included observation, audio-recording and analysis of multilingual classroom interaction, interviews with teachers, interviews with elite social actors who have been closely involved in the planning and implementation of language-in-education policy in the period following Independence in 2002. This paper takes a "longue durée" perspective, tracing the political, sociolinguistic and educational history of Timor-Leste. I argue for the need to chart out new directions in critical, ethnographic studies of language-in-education policies in the global south, incorporating perspectives on colonialism and coloniality, and taking account of local situated practices in schools and classrooms, along with the different language values and understandings of different social actors--values and understandings that have been shaped by wider political and ideological processes and by their positioning vis-á-vis these processes.

O artigo objetiva comparar e analisar as possibilidades de abordagem historiográfica de Ki-Zerbo e M’Bokolo a partir dos seus escritos sobre o Bilad es-Sudan. Esta pesquisa entende a acometida dos intelectuais enquanto homens do seu tempo. Sendo historiadores de gerações diferentes e apesar de poder-se nomeá-los num mesmo esforço de reconstrução de história da África, as suas perspectivas são distintas. Ki-Zerbo apresenta um olhar nacionalista, fato que, em grande medida, resulta da necessidade de confrontar as ideias da inferioridade histórica imputada pelo Ocidente a África; já M’Bokolo, propondo uma revisão do escrito de Ki-Zerbo, parte de uma perspectiva mais comprometida com novas disputas políticas e sociais das nações africanas.

One approach to identifying cancer-specific vulnerabilities and therapeutic targets is to profile genetic dependencies in cancer cell lines. Here, we describe data from a series of siRNA screens that identify the kinase genetic dependencies in 117 cancer cell lines from ten cancer types. By integrating the siRNA screen data with molecular profiling data, including exome sequencing data, we show how vulnerabilities/genetic dependencies that are associated with mutations in specific cancer driver genes can be identified. By integrating additional data sets into this analysis, including protein-protein interaction data, we also demonstrate that the genetic dependencies associated with many cancer driver genes form dense connections on functional interaction networks. We demonstrate the utility of this resource by using it to predict the drug sensitivity of genetically or histologically defined subsets of tumor cell lines, including an increased sensitivity of osteosarcoma cell lines to FGFR inhibitors and SMAD4 mutant tumor cells to mitotic inhibitors.

Abstract The present study investigated the effects of aerobic physical training on the femoral morphological, densitometric and biomechanical properties in growing male rats subjected to protein-based malnutrition. Four-week-old male Wistar rats were randomized into groups of 10 animals: Control Sedentary (CS), Control Trained (CT), Malnourished Sedentary (MS) and Malnourished Trained (MT). Control and malnourished animals received diets with 12% protein and 6% protein, respectively. The trained groups were submitted to a treadmill running program for 8 weeks. Total proteins and albumin were analyzed in the animals' blood plasma. Histological, densitometric and biomechanical analyzes were performed on the animals' femur. Body mass gain, physical performance, biochemical markers and the femoral morphological, densitometric and biomechanical properties were determined. Exercise tolerance increased in trained groups. Malnourished animals exhibited lower serum protein and albumin levels than controls. Porosity and trabecular bone density were not different between groups. The femoral maximum load, maximum load until fracture, resilience, stiffness, tenacity and densitometric properties were reduced by malnutrition. Physical training associated with malnutrition exacerbated the impairment in the femoral maximum load, maximum load until fracture, bone mineral content and density. Aerobic physical training worsens the damages induced by protein-based malnutrition in the femoral biomechanical and densitometric properties of growing male rats.

Os edifícios são colecionáveis? Houve quem colecionasse projetos, como o casal Tremaine, para quem Niemeyer e Johnson projetaram casas nunca construídas; certas exposições incorporaram protótipos arquitetônicos, como a casa que Breuer levantou no jardim do MoMA em 1949. Mas na grande maioria das vezes, as obras de arquitetura entram nos museus e na historiografia através de algo que as representa. Essas representações – fotografias, desenhos – geralmente compõem catálogos que, de certa maneira, desfrutam de uma vida independente das exposições que os originaram e superam-nas em longevidade. Latin American Architecture since 1945, catálogo decorrente da exposição homônima organizada por Henry-Russell Hitchcock para o MoMA em 1955, é hoje amplamente reconhecido como peça fundamental na construção historiográfica da arquitetura moderna latino-americana, bem como o Brazil Builds de Philip Goodwin (1943). Os catálogos de arquitetura são produtos bibliográficos onde as imagens têm papel preponderante sobre os textos. Entram na categoria da coleção de figuras ou do álbum fotográfico. Contudo, não obstante a prioridade da imagem sobre a palavra, o catálogo constitui, também, uma narrativa. O objetivo deste texto é interrogar a condição narrativa própria do catálogo como coleção de arquitetura, como álbum fotográfico, em suas correlações com os métodos da historiografia e com a formação do conceito de arquitetura moderna, através de Latin American Architecture since 1945.

This paper contributes to the growing field of family language policy by considering the life trajectories, language ideologies and linguistic investment of two transnational and multilingual families who have moved from Timor-Leste to the UK--to Dungannon, Northern Ireland. The paper draws on an ethnographic study conducted in Dungannon [da Costa Cabral, 2010. "From Dili to Dungannon: A case study of two bilingual migrant families from East-Timor" (Unpublished master's thesis). University of Birmingham]. The research included participant observation, field notes, language diaries, diary-based interviews and semi-structured interviews. It took as its starting point the view that migrant families are not static, self-contained units. Such families are situated within different social and historical contexts, and the language ideologies, repertoires and practices of family members are shaped by their lived experiences, over time, within these different contexts. The key insights gleaned in this study were as follows: The parents' beliefs and values regarding the languages in their communicative repertoires--Tetum, Portuguese and Indonesian--reflected their lived experiences in education and work, at different moments in the recent history of Timor-Leste. After moving to Northern Ireland, their language ideologies became closely bound up with their aspirations for their children. They encouraged their children's learning and use of English and did not show a strong commitment to the use of Tetum at home.

This article reveals how center-periphery relations have unfolded, over time, in language policy processes in one nation--Timor-Leste--on the global periphery. We take a "longue durée" perspective on the language policy processes at work in this historical context, showing how different regimes of language were imposed, in the past, by colonisers from distant centers--in Portugal and then in Java, Indonesia. Then, turning to the post-independence period, we show how a new order of indexicality, forged within the Resistance to the Indonesian occupation, formed the basis for current language policy in Timor-Leste, with Portuguese and Tetum as co-official languages. We also demonstrate that this agentive policy move, from the global periphery, oriented Timor-Leste to new and more complex center-periphery relations, to a "lusophone" world, with Portugal and Brazil as key players. Our account of contemporary policy discourses in Timor-Leste, and of the consequences for language policy implementation, on different scales (national and local), draws on recent research of an ethnographic and multi-scalar nature conducted in Timor-Leste (Da Costa Cabral, 2015).