Repositório RCAAP

O conhecimento da história das instituições e da sua interacção com o meio social em que se inseriram e actuaram depende muito da existência do seu arquivo. No entanto, quando as instituições não conservaram os seus sistemas de informação, por vicissitudes várias, as possibilidades de reconstituir a sua história ficam muito limitadas. Este pequeno trabalho tem como objectivo apresentar a tentativa da reconstituição de um sistema de arquivo pretérito a partir dos documentos sobreviventes conservados pelo produtor e por outras instituições que mantiveram com ele relações hierárquicas, temporais e associativas. Seleccionou-se a Santa Casa da Misericórdia de Sines, alvo de um estudo sistemático publicado este ano (Patrício, 2016), e um tempo em específico: a Época Moderna.

Esta Newsletter (NL) resulta de uma parceria entre o Instituto de Saúde Baseada na Evidência e a Cochrane Portugal, e tem como objectivo disponibilizar informação sobre áreas interessantes para a prática clínica, com base na melhor evidência científica. São incluídos estudos relevantes, criticamente avaliados pela sua validade, importância dos resultados e aplicabilidade prática, resumidos numa óptica de suporte à decisão. É dada prioridade a estudos de causalidade incluindo-se ainda, quando justificado, estudos qualitativos e metodológicos, assim como revisões científicas. O conteúdo da NL é da exclusiva responsabilidade do(s) seu(s) autor(es).

It has been shown that Notch signaling mediated by ligands of both Jagged and Delta families expands the hematopoietic stem cell compartment while blocking or delaying terminal myeloid differentiation. Here we show that Delta1- and Jagged1-expressing stromal cells have distinct effects on the clonogenic and differentiation capacities of human CD34(+) CD38(+) cells. Jagged1 increases the number of bipotent colony-forming unit-granulocyte macrophage (CFU-GM) and unipotent progenitors (CFU-granulocytes and CFU-macrophages), without quantitatively affecting terminal cell differentiation, whereas Delta1 reduces the number of CFU-GM and differentiated monocytic cells. Expression analysis of genes coding for Notch receptors, Notch targets, and Notch signaling modulators in supernatant CD34(+) cells arising upon contact with Jagged1 and Delta1 shows dynamic and differential gene expression profiles over time. At early time points, modest upregulation of Notch1, Notch3, and Hes1 was observed in Jagged1-CD34(+) cells, whereas those in contact with Delta1 strikingly upregulated Notch3 and Hes1. Later, myeloid progenitors with strong clonogenic potential emerging upon contact with Jagged1 upregulated Notch1 and Deltex and downregulated Notch signaling modulators, whereas T/NK progenitors originated by Delta1 strikingly upregulated Notch3 and Deltex and, to a lesser extent, Hes1, Lunatic Fringe, and Numb. Together, the data unravel previously unrecognized expression patterns of Notch signaling-related genes in CD34(+) CD38(+) cells as they develop in Jagged1- or Delta1-stromal cell environments, which appear to reflect sequential maturational stages of CD34(+) cells into distinct cell lineages.

O presente trabalho de investigação aplicada centra-se na temática da equivalência intercultural, e consiste no estudo de uma bateria de testes de aptidões, utilizada em diversas organizações nacionais e internacionais, em contextos de avaliação psicológica – Bateria de Raciocínio Crítico (CRTB). Partiu do pressuposto de que as versões originais e as versões adaptadas de cada teste são equivalentes, e procedeu à comparação entre as versões do Reino Unido (original) e de Portugal, da Austrália e da África do Sul (adaptações), e de Portugal (original) e Moçambique (adaptação). Partindo de um total 4946 respostas a três testes de aptidões - Verbal, Numérico e Diagramático - foi efectuado o estudo do Funcionamento Diferencial dos Itens (DIF), nas versões aplicadas nos cinco países, com recurso a metodologia baseada na Teoria da Resposta ao Item (TRI), nomeadamente, pela aplicação do Modelo de Rasch. Detectou-se a presença de DIF em alguns itens, nos vários testes, o que pode comprometer a equivalência intercultural das diferentes versões. Contudo, a análise efectuada não permitiu concluir que os testes são, na sua globalidade, culturalmente enviesados, devido a limitações na qualidade dos dados amostrais disponíveis. Este estudo alertou para a necessidade de averiguar a qualidade dos conteúdos dos itens e para a conveniência da utilização conjunta de Métodos Estatísticos e dos Juízes, nas adaptações de testes, assim como para a necessidade de interpretação cautelosa, em decisões de selecção que tenham por base os resultados de instrumentos psicométricos adaptados. Adicionalmente, reuniu evidências das potencialidades e vantagens da metodologia TRI para a investigação em Psicologia Intercultural.

Cell migration is an important biological process which has been intensively studied in the past decades. Numerous techniques, mainly involving two-dimensional cell culture systems, have contributed to dissecting the essential mechanisms underlying this process. However, the development of three-dimensional cell culture and in vivo systems has shown some differences with what was previously believed to be well-established cell migration mechanisms, suggesting that two-dimensional cell motility would be a poor predictor of in vivo behaviour. Drosophila is a widely recognized model organism to study developmental and homeostatic processes and has been widely used to investigate cell migration. Here, we focus on the migration of small groups of pupal hemocytes that accumulate during larval stages in dorsal patches. We show that integrins, and other known nascent adhesion-related proteins such as Rhea and Fermitin 1, are crucial for this process and that their depletion does not affect polarization in response to environmental cues. We also present evidence for the importance of adhesion maturation-related proteins in hemocyte migration, namely Zyxin. Zyxin depletion in hemocytes leads to a significant increase of cell speed without affecting their response to a chemotactic cue. This is the first report of a systematic analysis using Drosophila melanogaster hemocytes to study adhesion-related proteins and their function in cell migration in vivo. Our data point to mechanisms of cell migration similar to those described in three-dimensional in vitro systems and other in vivo model organisms.

Background: Cancer aetiology is multifactorial and risk factors include: obesity, central adiposity, sedentarism, excessive or deficient intake of foods and/or nutrients with pro-carcinogenic effects vs protective ones. Objectives: To evaluate the pattern of nutritional status, life styles, physical activity and diet in a cohort of cancer patients. Methods: This pilot cross-sectional study was conducted in 64 patients referred for radiotherapy at the Radiotherapy Department of the University Hospital of Santa Maria (CHLN). Evaluations were: waist circumference associated with potential cardio-metabolic risk, body composition by Tetrapolar Bioimpedance Analysis (XITRON®), Body Mass Index, dietary intake pattern with a short food frequency questionnaire, physical activity with Jackson questionnaire. Results: The most frequent diagnosis were breast and colorectal cancers; 53% of patients were overweight/obese, and there was a significant correlation between this nutritional pattern and weight gain in comparison with usual weight (p<0.005). There were 78% of patients with a waist circumference above the maximum cut-off limit, indicating moderate/ high cardio-metabolic risk, and most were female patients (87%). The great majority of patients (61%) had excessive fat mass highly above the maximum recommended cut-off value, especially male patients (74%). The dietary pattern was poor in vegetables (55%) and excessive in meat and simple carbohydrates (78%); physical activity was low with a high prevalence of sedentarism. Conclusions: This population presented excessive body weight, excessive fat mass, high cardio-metabolic risk, sedentarism and an unbalanced diet poor in protective foods/nutrients. This population's life styles and nutritional pattern, may be considered of risk in oncology disease. The elevated and growing incidence of cancer in Portugal, reinforces the need for further research in order to identify nutritional factors involved in the etiology/evolution and probably prognosis of cancer.

Background: Cavotricuspid isthmus (CTI) ablation in typical atrial flutter (AFL) restores sinus rhythm in 95% of patients, which may lead to the discontinuation of oral anticoagulation during follow-up. Therefore, we aimed to systematically review the clinical impact of oral anticoagulation in the incidence of thromboembolic events (TE) after typical AFL ablation. Methods: We searched for controlled studies evaluating the impact of anticoagulation in the incidence of TE in patients submitted to AFL ablation in MEDLINE, CENTRAL, PsycINFO database (June/2021). The primary outcome was TE events (ischemic stroke or systemic embolism). A meta-analysis was performed deriving risk ratios (RR) and 95% confidence intervals (CI). Statistical heterogeneity was measured through I2 metric. The confidence in the evidence was appraised with Grading of Recommendations Assessment, Development and Evaluation (GRADE) framework. Results: Eight observational studies with 4870 patients were included. TE events were not significantly reduced (RR 1.18, 95% CI 0.59-2.36; n = 4870; GRADE very low). A meta-regression showed that for each 10% increase in the prevalence of previous AF in the studied population, anticoagulation reduced TE risk in 32%. There were no significant differences regarding bleeding events (RR 2.16, 95% CI 0.43-10.97, I2 = 0%; GRADE low), but there was a lower all-cause mortality (RR 0.24, 95% CI 0.17-0.32, GRADE low). Conclusion: The best available evidence lacks robustness and the data did not definitely associate anticoagulation after typical AFL ablation with reduced TE.

Resilience is a dynamic, multidimensional complex concept that implies risk and protective factors, adaptation, and success. Communication and language are often identified as barriers in deaf children's development. However, research linking deafness in childhood and resilience is scarce. The present comprehensive literature review aims to verify which are the predominant risk factors for this group, which protective factors may be identified and if significant differences have been found between deaf and hearing children regarding resilience. A systematic search, performed in seven databases, identified 11 articles published in peer-reviewed journals between 2000 and 2019 that met the criteria. Deaf children experience exposure to risk through obstacles in communication, language, and information failure. Consequently, differences between hearing and deaf children are related to more difficulties in emotion regulation and interpersonal relationships. Principal protective factors are a supportive family, school staff, and peers. Practical implications and recommendations for future research are provided.

O projeto Gama é um projeto de resistência que contrapõe, de modo consistente, discursos novos, que colocam em contacto novos intervenientes, novas agências, novas afinidades. É um campo de consolidação cultural que reúne os 15 autores aqui representados. O contexto é reconhecido como de viragem educativa , quando se observa uma tendência de convergência de agências na direção inclusiva, colaborativa, participativa, por parte de instituições, escolas, museus, plataformas de disseminação e de produção, artistas e educadores. O projeto Gama, e com ele, o projeto do Congresso CSO (Criadores sobre outras Obras), é um projeto que reivindica uma resistência para contrapor, de modo consistente, discursos novos, colocando em contacto novos intervenientes, gerando novas agências, favorecendo afinidades. O projeto é simples: é preciso dar a conhecer quem está por conhecer, é preciso fazer funcionar os circuitos relacionais excêntricos e antepor à colaboração um conhecimento mútuo que robustece os discursos, e origina novidade nos discursos sobre arte.

Arte, vaidade, um novo olhar. Se há coisa que a arte inclua na sua definição é a sua vaidade: os homens morrem, a sua arte permanece, é cuidada, merece ser conhecida, ensinada, mostrada, protegida, divulgada. Neste propósito se justifica o projeto GAMA: há obras em risco de desconhecimento, outras que precisam de voltar a ser olhadas, outras ainda que não são conhecidas. A plataforma do Congresso CSO tem permitido uma valorização e disseminação de muitos autores. As afinidades vão-se desenhando. Aqui está enunciado um projeto de formação artística não formal: criação de públicos, disseminação, geração de interações, promoção de conhecimentos, deslocações, construções, interrogações.

Recentemente foi avaliada em consulta domiciliaria de medicina geral e familiar uma doente hipertensa de 94 anos, com diversas comorbilidades e polifarmácia, que apresentou análises com hiponatrémia (127 mmol/L), tendo já sido internada há um mês por prostração (sódio de 110 mmol/L). A desprescrição do diurético tiazídico (hidroclorotiazida 25 mg) corrigiu progressivamente a natremia, mantendo-se a pressão arterial controlada.

Introduction: Immunity against measles may result from previous contact with the virus or vaccination. In this study we aimed to evaluate the prevalence of immunity to measles in healthcare professionals of a central hospital. Material and methods: Retrospective study, with description of the results of measles-specific IgG assay in healthcare professionals, between May 2010 and March 2018. Results: The results of 1339 healthcare professionals were analyzed. The average age was 39.3 ± 10.11 years, 71.1% female. The prevalence of positive IgG was 81.5%, higher among professionals in the age groups 40 - 49 and over 50 years (91.9% and 94.6% respectively). Healthcare professionals who presented negative or equivocal IgG were mostly under 40 years old (83.1%) (p < 0.05). Discussion: The prevalence of serological immunity to measles in healthcare professionals was found to be lower than in other studies. That may be due to differences between the characteristics of the studied samples. Susceptibility to measles was higher in lower age groups. These results may reflect low vaccination coverage in this age group, an incomplete vaccination schedule, or the possibility of older healthcare professionals having the disease, which may confer an immune response with higher IgG levels. Conclusion: Knowing the prevalence of susceptibility to measles in healthcare professionals enabled the establishment of prevention strategies for outbreaks that may occur. Vaccination remains the best preventative measure, but a third dose of vaccine may be considered in certain epidemiological contexts, particularly in the youngest exposed healthcare professionals.

Background: There is limited knowledge on health-related quality of life (HRQoL) in late-stage Parkinson's disease (PD). Objective: To assess factors associated with HRQoL in patients with late-stage PD, with a focus on health care provision. Methods: The Care of Late Stage Parkinsonism (CLaSP) project is the largest study on late-stage PD to date. The current study analyzed data of 401 patients from 6 European countries in whom HRQoL was assessed with the 8-item PD Questionnaire in patients without dementia. Factors potentially associated with HRQoL were assessed and examined in linear regression analyses. Results: Better HRQoL was associated with living at home, greater independence in activities of daily living (Schwab and England Scale), less severe disease (Hoehn and Yahr stage), better motor function (Unified PD Rating Scale Part III), and lower non-motor symptoms burden (Non-Motor Symptoms Scale [NMSS]) across all NMSS domains. Having a PDspecialist as physician for PD, contact with a PDnurse, and no hospital admission during the past 3 months were associated with better HRQoL, but having seen a physiotherapist or occupational therapist was associated with worse HRQoL. Conclusions: The results emphasize the importance of optimizing treatment for motor and multiple non-motor symptoms to improve HRQoL in patients with late-stage PD. PD-specific health care resources, particularly PDnurses, are likely important in addressing issues to improve HRQoL in this population. Worse HRQoL in those who had recently seen a physiotherapist or occupational therapist may reflect referral based on factors not measured in this study.

O objectivo da presente investigação prende-se com o nosso interesse em 1) estudar a relação entre culpa e vergonha e as dimensões anaclítica e introjectiva da personalidade; e 2) estudar a relação entre culpa e vergonha e a sintomatologia depressiva. Para atingir estes objectivos formulámos três hipóteses de investigação. A primeira estabelece que a correlação entre a culpa e a dimensão introjectiva da personalidade é mais elevada do que a correlação entre a culpa e a dimensão anaclítica da personalidade. A segunda hipótese estabelece que a correlação entre a vergonha e a dimensão anaclítica da personalidade é mais elevada do que a correlação entre a vergonha e a dimensão introjectiva da personalidade. Por fim, a terceira hipótese estabelece que a correlação entre a culpa e a sintomatologia depressiva é mais elevada do que a correlação entre a vergonha e a sintomatologia depressiva. Participaram na investigação 145 estudantes de duas instituições de Ensino Superior da cidade de Lisboa com idades compreendidas entre 19 e 47 anos. Todos os participantes responderam a um questionários sobre dados sócio-demográficos, à versão portuguesa do Questionário de Experiências Depressivas, à tradução portuguesa do Test of Self- Conscious Affect e à versão portuguesa da Escala de Depressão do Centro de Estudos Epidemiológicos. Os resultados obtidos não confirmam as hipóteses formuladas, mas foi verificado: 1) a ausência de correlação entre culpa e a dimensão introjectiva da personalidade mas correlação positiva, moderada e significativa entre culpa e dimensão anaclítica da personalidade; 2) correlação positiva moderada e significativa entre vergonha e as dimensões anaclítica e introjectiva da personalidade; e 3) uma correlação mais elevada entre vergonha e sintomatologia depressiva do que entre culpa e sintomatologia depressiva.

Muscular cramp is a common symptom in healthy people, especially among the elderly and in young people after vigorous or peak exercise. It is prominent in a number of benign neurological syndromes. It is a particular feature of chronic neurogenic disorders, especially amyotrophic lateral sclerosis. A literature review was undertaken to understand the diverse clinical associations of cramp and its neurophysiological basis, taking into account recent developments in membrane physiology and modulation of motor neuronal excitability. Many aspects of cramping remain incompletely understood and require further study. Current treatment options are correspondingly limited.

The novel coronavirus disease-2019 (COVID-19), caused by the pathogen severe acute respiratory syndrome-CoV-2, is causing a global pandemic, with over 26.9 million cases and 880,000 deaths as of September 6, 2020. While there has been speculation and observational research about the impact of COVID-19 on asthma, much remains unknown. The goal of this article is to provide a scoping review on pediatric asthma and COVID-19 and summarize what we do and do not know from the first wave of the pandemic.

Parkinson's disease (PD) is a progressive neurodegenerative disease. The currently available treatment options only have a symptomatic effect. With disease progression almost all antiparkinsonian pharmacological classes are tried, but the gold standard of pharmacological management is still L-dopa. Various strategies can be used to raise the dopaminergic tone. Catechol-O-methyltransferase (COMT) inhibitors attain this goal by decreasing L-dopa peripheral metabolism. Areas covered: Opicapone (Ongentys®) is a new COMT inhibitor developed to fulfill the need for more potent, safer and longer acting COMT inhibitors. This review puts into context opicapone's indications, its chemical and preclinical data, the pharmacodynamics and pharmacokinetic characteristics, and the efficacy and safety results delivered by clinical trials. Expert opinion: Opicapone is an efficacious COMT inhibitor. Its proprieties make it adequate for a once-a-day oral dose regimen. It has proved to reduce the off-time and to increase the on-time without troublesome dyskinesias in PD patients with motor fluctuations. The reported adverse events suggest an overall safe and well-tolerated profile. The most common adverse events were dyskinesia, and there were no issues of concern for hepatotoxicity, severe diarrhoea or chromaturia. Further evidence is still needed to conclude how it compares with other drugs for the treatment of motor fluctuations.

Objective: To summarize the 2010 EFNS/MDS-ES evidence-based treatment recommendations for the management of Parkinson's disease (PD). This summary includes the treatment recommendations for early and late PD. Methods: For the 2010 publication, a literature search was undertaken for articles published up to September 2009. For this summary, an additional literature search was undertaken up to December 2010. Classification of scientific evidence and the rating of recommendations were made according to the EFNS guidance. In cases where there was insufficient scientific evidence, a consensus statement ('good practice point') is made. Results and conclusions: For each clinical indication, a list of therapeutic interventions is provided, including classification of evidence.

Clinical Trials Corner of Journal of Huntington’s Disease will regularly review ongoing and recently completed clinical trials in Huntington’s disease. In this inaugural issue, we list all currently registered and ongoing clinical trials, expand on LEGATO-HD and IONIS-HTTRx, and cover two recently finished trials: Amaryllis and Pride-HD.

In recent years an increased level of motor neuronal excitation has been proposed as important in the neuronal degeneration so characteristic of amyotrophic lateral sclerosis (ALS/MND) (Van Den Bosch et al., 2006). This notion derives from the finding that levels of glutamate, an excitatory neurotransmitter, are raised in the cerebrospinal fluid in about 20% of patients with ALS/MND (Plaitakis and Caroscio, 1987) and glutamate transport is abnormal in brain and spinal cord tissue at autopsy (Rothstein et al., 1992; Yamanaka and Komine, 2018). Further, measurements of short interval cortical inhibition (SICI) in ALS/MND, using a threshold tracking protocol involving paired pulse transcranial magnetic stimulation of motor cortex, have shown that increased cortical excitability, determined by reduced cortical inhibition, may precede the clinical onset of ALS, and that this becomes more marked as the disease progresses (Vucic et al., 2008; Shibuya et al., 2017).