ABSTRACT Sarcopenia is a progressive age‑related loss of muscle mass associated with a decline in muscle function and physical performance. Patients with chronic kidney disease experience substantial loss of muscle mass, weakness, and poor physical performance. Indeed, with the progression of chronic kidney disease, skeletal muscle dysfunction contributes to mobility limitation, loss of functional independence, and vulnerability to disease complications. There is a lack of robust data on the negative effect of the impact of kidney disease on skeletal muscle dysfunction, as well as on screening and treatment strategies that can be used in clinical practice to prevent functional decline and disability. Therefore, sarcopenia may be na underestimated condition with major implications for people with chronic kidney disease, even before the start of dialysis, which makes research into this topic necessary. The purpose of this review is to expand on some fundamental topics of sarcopenia, with an emphasis on the setting of chronic kidney disease patients.

Resumen Introducción: La simulación permite a los estudiantes, a través de la creación de escenarios hipotéticos, desarrollar habilidades, haciéndolos más activos en el proceso de toma de decisiones en relación al cuidado de enfermería. Objetivo: Evaluar la efectividad de la simulación en el aprendizaje de estudiantes de pregrado y posgrado de enfermería. Métodos: Estudio cuantitativo, descriptivo y exploratorio en el que participaron 233 estudiantes, de los cuales 164 son del curso de pregrado en enfermería y 69 son de educación de posgrado. Como método de recolección de datos, se utilizó un inventario llamado "El Inventario de Simulación de Efectividad de Aprendizaje", después de la traducción al português. El análisis de los datos se realizó con el sistema operativo SPSS® versión 23. Resultados: Los participantes del estudio tuvieron una respuesta promedio superior en todas las variables evaluadas: curso, recurso, información, habilidades clínicas, confianza, resolución de problemas, colaboración, preparación y resultado. Conclusión: La simulación demuestra ser una metodología de enseñanza eficaz en los cursos de pregrado y posgrado en enfermería.

Resumo Introdução: A simulação permite aos estudantes, através da criação de cenários hipotéticos, desenvolverem competências, tornando-os mais ativos no processo de tomada de decisão face aos cuidados de enfermagem. Objetivo: Avaliar a eficácia da simulação na aprendizagem dos estudantes do curso de licenciatura e ensino pós-graduado de enfermagem. Métodos: Estudo quantitativo, descritivo e exploratório onde participaram 233 estudantes, dos quais 164 são do curso de licenciatura em enfermagem e 69 são do ensino pós-graduado. Como método de recolha de dados foi utilizado um inventário intitulado como “The Simulation Learning Effectiveness Inventory”, após tradução para português. A análise dos dados foi realizada através do sistema operativo SPSS® versão 23. Resultados: Os participantes do estudo apresentaram valores médios elevados em todas as variáveis avaliadas: curso, recurso, debriefing, habilidades clínicas, confiança, resolução de problemas, colaboração, preparação e resultado. Conclusão: A simulação revela-se uma metodologia de ensino eficaz nos cursos de licenciatura e ensino pós-graduado de enfermagem.

Abstract Introduction: Simulation allows students, through the creation of hypothetical scenarios, to develop skills, making them more active in the decision-making process in relation to nursing care. Objetive: To evaluate the effectiveness of simulated practices in the learning of undergraduate and graduate nursing students. Methods: Quantitative, descriptive and exploratory study in which 233 students participated, of which 164 are from the nursing undergraduate course and 69 are from postgraduate education. As a method of data collection, an inventory called “The Simulation Learning Effectiveness Inventory” was used, after translate to Portuguese language. Data analysis was performed using the SPSS® version 23. Results: Study participants had a higher average response in all variables evaluated: course, resource, debriefing, clinical skills, confidence, problem solving, collaboration, preparation and result. Conclusion: Simulation proves to be an effective teaching methodology in undergraduate and postgraduate nursing courses.

RESUMO A doença de lesões mínimas é responsável por até 25% dos casos de síndrome nefrótica na população adulta. O mecanismo alérgico tem sido associado à doença de lesão mínima a associada a alérgenos, como picadas de insetos. Apresentamos um caso de uma mulher com início recente de síndrome nefrótica após picada de inseto não himenóptero, com doença de lesões mínimas comprovada por biópsia, acompanhada por um processo de tromboembolismo pulmonar. A paciente teve remissão completa com glicocorticoides, com resposta sustentada por 6 meses após a interrupção do tratamento. Uma nova exposição à picada de inseto na mesma região geográfica e estação do ano provocou uma recaída da síndrome nefrótica. Evitar o local subsequentemente resultou em remissão sustentada por mais de 4 anos.

ABSTRACT Minimal change disease accounts for up to 25% of the cases of nephrotic syndrome in adult population. The allergic mechanism has been associated with minimal change disease and allergens have been implied, namely insect stings. We present a case report of a woman with new onset of nephrotic syndrome after a non-hymenoptera insect sting, with biopsy-proven minimal change disease, that was accompanied by a pulmonary thromboembolism process. Complete remission with glucocorticoid therapy was observed, with sustained response for 6 months after discontinuation. A new exposure to insect sting in the same geographical region and season triggered a nephrotic syndrome relapse. Subsequent avoidance of the place resulted in a sustained remission for more than 4 years.

Resumo Uma das causas mais comuns da glomerulonefrite rapidamente progressiva (GNRP) é a glomerulonefrite crescêntica (GNC) pauci-imune. Na maioria dos casos, a patologia apresenta um marcador sorológico positivo, o anticorpo anticitoplasma de neutrófilos (ANCA), mas em cerca de 10% dos pacientes não há ANCAs circulantes, perfazendo um subgrupo da patologia conhecido como GNC pauci-imune ANCA-negativa. A GNRP pode estar associada a doenças sistêmicas, mas são poucos os relatos de caso que descrevem sua associação com doença mista do tecido conjuntivo (DMTC). O presente artigo relata um caso de GNC ANCA-negativa associada a DMTC.

Abstract One of the most common causes of rapidly progressive glomerulonephritis (RPGN) is pauci-immune crescentic glomerulonephritis (CrGN). In the majority of cases, this condition has a positive serologic marker, the anti-neutrophil cytoplasmic antibodies (ANCAs), but in approximately 10% there are no circulating ANCAs, and this subgroup has been known as the ANCA-negative pauci-immune CrGN. RPGN can be associated with systemic diseases, but there are only few case reports describing the association with mixed connective tissue disease (MCTD). The authors report a case of ANCA-negative CrGN associated with a MCTD.

Este artigo centra-se na representação de homens e mulheres nas engenharias em Portugal, tanto ao nível da educação como do exercício da profissão de engenheiro/a. Evidencia-se que a engenharia permanece essencialmente representada por homens, sendo a designação no plural (engenharias) aquela que mais se adequa ao panorama nacional. Há áreas da engenharia onde a proporção do sexo feminino supera a do sexo masculino, e outras onde prevalece um padrão de hegemonia masculina. Os processos de socialização genderizados continuam a moldar as opções educativas de rapazes e raparigas, condicionando as suas aspirações e oportunidades profissionais e contribuindo para a reprodução da segregação sexual das profissões.

This article focuses on the representation of men and women in engineering in Portugal, both at the tertiary level of education and professional practice. A key conclusion is that despite the gender imbalances and the overrepresentation of men, the adoption of the plural form (engineering fields) is the most adequate one to describe the current national context. There are domains where women outnumber men, whereas other still prevail as male-dominated areas. Gendered socialisation processes are still shaping the educational options of boys and girls, therefore constraining their career aspirations and opportunities, as well as reproducing gender-based occupational segregation.

ABSTRACT Hepatitis C virus (HCV) is widely prevalent worldwide, with an estimated 180 million people infected. Its manifestations are not limited to hepatic disease, as it has widely known immunomodulator and oncogenic effects. Most HCV associated autoimmune and lymphoproliferative diseases have been shown to subside after successful antiviral treatment. However, viral eradication doesn't always equal lymphoma cure. In some cases, the oncogenic effect lasts well beyond viral elimination. Although the new direct anti-viral agents (DAA) have proved to be safe and effective in treating HCV (with sustained viral responses of 91-95%), this treatment has inherent toxicities. Further, immunologic manifestations of HCV-associated diseases, when present, may require broad immunosuppression in association with the antiviral treatment. We present a case that illustrates the complexities of treating these viral mediated immunologic and lymphoproliferative diseases

RESUMO A doença renal ateroembólica (DRAE) é uma manifestação renal da aterosclerose enquanto patologia sistêmica. A DRAE é definida como uma disfunção renal secundária à embolização de cristais de colesterol seguida da oclusão da vascularização renal. O presente relato descreve o caso de um paciente com vários fatores de risco, porém sem um evento precipitante, que se apresentou com um curso clínico bastante atípico de doença ateroembólica grave de evolução espontânea e silenciosa.

ABSTRACT Atheroembolic renal disease (AERD) is a kidney manifestation of atherosclerosis as a systemic disease. AERD is defined as a renal impairment secondary to embolization of cholesterol crystals with consequent occlusion of renal vascularization. The current case report describes one patient with multiple risk factors but without any inciting event history who presents a very atypical clinical course of a severe and massive atheroembolic disease that developed spontaneously and silently.

Among thrombotic microangiopathies (TMA), the hemolytic uremic syndrome associated with dysregulation of the alternative complement pathway (aHUS) is one of the most challenging diseases a nephrologist can face. By the end of the XXth century, the complement’s role was unraveled with the discovery that mutations in the factor H coding gene were responsible for aHUS. But it was the acknowledgment that pharmacological C5-9 blockage provided a cure for aHUS that fostered the interest of the nephrology community in the genetics, pathophysiology and therapeutics of, not only of aHUS, but TMA in general. The molecular genetics of aHUS is technically demanding and, as such, in Portugal (alike many other European countries) a single laboratory emerged as a national reference center. The fact that all samples are evaluated in a single center provides a unique opportunity for data collection and a forum for discussion for all those interested in the field: immunologists, molecular geneticists, pathologists and nephrologists. The current consensus document emerged from such a discussion forum and was sponsored by the Portuguese Society of Nephrology. The goal is more to portray the Portuguese picture regarding the diagnostic approach and therapeutic options than to extensively review the state of the art of the subject. The accompanying documents that are published as supplementary data are in line with that goal. They range from the informed consent and clinical form to be sent together with the biological samples for genetic testing, to the appendix regarding the actual sampling and storing conditions. The document is also intended to set an example for future documents and independent discussion forums on other kidney diseases for which emerging diagnostic and/or therapeutic strategies are reaching clinical practice

Introduction: Improved technique and materials have allowed us to prolong the life of hemodialysis vascular access using percutaneous transluminal balloon angioplasty (PTA). Central vein stenosis (CVS) can lead to arteriovenous access dysfunction or thrombosis. Our goal was to revise the outcomes of our institution, evaluating the immediate and long-term results in the endovascular treatment of CVS. Methods: We reviewed the data of all procedures performed in our center, Centro Hospitalar e Universitário de Coimbra, during a five-year period July 2009 and June 2014, selecting the cases that had a CVS diagnosis. We evaluated the immediate result and the existence of complications during the procedure. Long-term evaluation of PTA results of the 26 patients with a successful PTA was made through contact with the referring hospital or hemodialysis clinic. Primary and assisted access patencies were verified retrospectively at 3, 6, 12 and 24 months post-intervention. Results: Of the 31 patients in whom there were an intention to treat, in 5 the stenosis was in fact an occlusion and the guide wire could not be passed. The remaining 26 patients underwent PTA with improvement/resolution of the lesion. Consequently, we had an initial intervention success rate of 83.9%. Minor complications occurred in 2 patients. The long-term follow-up results were primary patency at 3, 6, 12 or 24 months of 88%, 63%, 31% and 6%, respectively, and assisted primary patency at 3, 6, 12 or 24 months of 88%, 76%, 70% and 46%, respectively. Conclusion: CVS is a common problem in hemodialysis patients. Our center results are consistent with current literature and demonstrate the benefit of PTA with excellent immediate success. However, the high recurrence rate of these stenoses requires in many cases multiple PTA, with low long-term primary patency.

RESUMO: A pesquisa teve como objetivo desenvolver um trabalho compartilhado com professores da educação básica sobre questões socioambientais locais e analisar o processo de interação, reflexão e elaboração pelos sujeitos participantes da pesquisa, cujo tema mobilizador teve foco na conservação e na preservação de nascentes. Os instrumentos de coleta de dados foram: audiogravação de todos os encontros, diário de campo da pesquisadoras, questionários abertos e registros escritos pelos professores. A análise dos dados decorrente dos encontros se baseou na metodologia denominada Análise de Conteúdo. Observamos uma maior interação dos professores com o tema da pesquisa quando eles se apropriaram do tema e o relacionaram com a problemática socioambiental local.

ABSTRACT: This research aimed to develop a shared work with basic education teachers about local socio-environmental issues and analyze the process of interaction, reflection and preparation by subjects participating in the survey. The subject's mobilizing theme was focused on conservation and preservation of springs. The tools used for data collection were: audio recording of all the meetings, field diary of the researcher, open questionnaires and reports written by the teachers. The analysis of data from the meetings was based on the methodology called Content Analysis. We observed teachers' higher interaction with the theme of the research when they made the theme relevant and related it to their local socio-environmental issue.

RESUMEN: La investigación tiene el objetivo de desarrollar un trabajo compartido con los profesores de educación básica acerca de cuestiones socio-ambientales locales y analizar el proceso de interacción, reflexión y elaboración por los sujetos participantes de la investigación, cuyo tema movilizador tuvo foco en la conservación y la preservación de nacientes. Los instrumentos de colección de datos fueron: audio-grabación de todos los encuentros, diario de campo de la investigadora, cuestionarios abiertos y registros escritos por los profesores. El análisis de los datos que partieron de los encuentros se basó en la metodología del Análisis de Contenido. Observamos interacción más grande entre los profesores con el tema de la investigación cuando ellos se apropiaron del tema y lo relacionaron con la problemática socio-ambiental local.

Após duas décadas de intensa investigação no domínio da genética e da fisiopatologia da doença poliquística renal autossómica dominante foram publicados os resultados de alguns ensaios clínicos aleatorizados e controlados envolvendo milhares de doentes. Neste artigo os autores revêm e discutem os resultados clínicos, os efeitos adversos e as limitações destes estudos bem como antevêm as futuras linhas de investigação neste domínio.

After two decades of major achievements in the understanding of genetics and pathophysiology of autosomal dominant polycystic kidney disease (ADPKD) several Stage III randomized and controlled clinical trials involving thousands of patients had the results published recently. In this article, the authors review the results of the major clinical trials discussing the clinical outcomes, adverse effects and limitations as well as the future directions in this exciting investigation.

El desarrollo de la Terapia de Remotivacióon en la intervención gerontológica, a pesar de ser reciente, ha demostrado tener un impacto positivo tanto en pacientes mayores con demencia leve con alguno deterioro funcional y cognitivo, como en mayores autónomos e independientes. Los experimentos reportados señalan una mejora de la interacción entre individuos, lo que contribuye a su integración y aumento de la autoestima. Este estudio procura analizar los beneficios de un Programa de Terapia de Remotivación para adultos mayores institucionalizados (ProTR) en lo que concierne los niveles de sintomatología depresiva, ánimo y participación. Hubo 14 articipantes, divididos en dos grupos: el experimental (que participó en las sesiones del ProTR) y el grupo de control (sin intervención). Los instrumentos utilizados fueron la Escala Geriátrica de Estado del Ánimo, la Escala de Depresión Geriátrica y la Escala de Evaluación del Comportamiento en la Remotivación. Los resultados sugieren beneficios en la disminución de los síntomas depresivos después de la intervención y en una mejora del estado del ánimo en el follow-up. Los beneficios identificados en este estudio piloto justifican un mayor desarrollo y evaluación de este programa en el futuro, incluyendo a otras poblaciones.

O desenvolvimento da Terapia de Remotivação na intervenção gerontológica, apesar de recente, tem vindo a demonstrar um impacto positivo, tanto em idosos com demência leve com algum comprometimento funcional e cognitivo, como em idosos autónomos e independentes. As experiências reportadas apontam para uma melhoria da interação entre indivíduos, o que contribui para a sua integração social e aumento da autoestima. Este artigo procura analisar os benefícios de um Programa de Terapia de Remotivação para idosos institucionalizados (ProTR), aos níveis da sintomatologia depressiva, ânimo e participação. Os sujeitos foram 14, divididos em duas condições: grupo experimental (que participou nas sessões do ProTR) e grupo de controlo (sem intervenção). Os instrumentos utilizados foram a Escala Geriátrica de Ânimo, a Escala de Depressão Geriátrica e a Escala de Avaliação do Comportamento na Remotivação. Os resultados obtidos sugerem que o ProTR contribuiu para a diminuição da sintomatologia depressiva após a intervenção e para o aumento do ânimo no follow-up. Os benefícios encontrados neste estudo piloto justificam o futuro desenvolvimento e avaliação deste programa, nomeadamente junto de outras populações.

The development of Remotivation Therapy in gerontological interventions is recent but has shown a positive impact in patients experiencing mild dementia, with some functional and cognitive impairment, as well as in autonomous and independent elderly. Reported experiences indicate an improvement in interactions between individuals, which contributes to their social integration and increased self-esteem. This pilot study analyzes the benefits of a Remotivation Program for institutionalized elderly (ProTR) in terms of depressive symptoms, morale and participation. The participants were 14 seniors divided into two groups: an experimental group who benefited from ProTR sessions and a control group who experienced no intervention. Instruments used include the Philadelphia Geriatric Centre Morale Scale, the Geriatric Depression Scale and the Scale for the Evaluation of the Behaviour in Remotivation. The results suggest benefits in terms of depressive symptom reduction after the intervention, and an increase in morale at follow-up. The authors highlight the need for more research and evaluation of this intervention with other population groups.

Metalloproteinases are abundant enzymes in crotaline and viperine snake venoms. They are relevant in the pathophysiology of envenomation, being responsible for local and systemic hemorrhage frequently observed in the victims. Snake venom metalloproteinases (SVMP) are zinc-dependent enzymes of varying molecular weights having multidomain organization. Some SVMP comprise only the proteinase domain, whereas others also contain a disintegrin-like domain, cysteine-rich, and lectin domains. They have strong structural similarities with both mammalian matrix metalloproteinases (MMP) and members of ADAMs (a disintegrin and metalloproteinase) group. Besides hemorrhage, snake venom metalloproteinase induce local myonecrosis, skin damage, and inflammatory reaction in experimental models. Local inflammation is an important characteristic of snakebite envenomations inflicted by viperine and crotaline snake species. Thus, in the recent years there is a growing effort to understand the mechanisms responsible for SVMP-induced inflammatory reaction and the structural determinants of this effect. This short review focuses the inflammatory effects evoked by SVMP.