Head-Up Tilt Testing with Different Nitroglycerin Dosages: Experience in Elderly Patients with Unexplained Syncope

AIMS: Protocols using sublingual nitrates have been increasingly used to improve diagnostic accuracy of head-up tilt testing (HUT). Nevertheless, exaggerated responses to nitrates have been frequently described, particularly in elderly patients. The aim of this article is to evaluate, in an elderly population with unexplained syncope, whether the impact of sublingual nitroglycerin (NTG) used as a provocative agent is dose-dependent. METHODS AND RESULTS: One hundred and twenty consecutive elderly patients submitted to HUT using NTG after an asymptomatic drug-free phase were studied. Patients were divided into three groups according to the NTG dosage: 500, 375 and 250 microg. The test was considered positive when there was reproduction of symptoms with bradycardia and/or arterial hypotension. A gradual decrease in the blood pressure after NTG was considered an exaggerated response to nitrates. There were no differences in the clinical characteristics of the different subgroups. A positive test was obtained in 50% of the patients in each group. The rate of exaggerated responses was identical in all groups and ranged between 15 and 17%. CONCLUSION: In an elderly population with syncope of unknown origin submitted to HUT, the response to NTG is not dose-dependent, and no difference was found in the rate of exaggerated responses to nitrates with different NTG dosages.

Lupus and Pregnancy. 15 Years of Experience in a Tertiary Center

This retrospective study was designed to evaluate the outcome of pregnancies in women diagnosed with systemic lupus erythematosus (SLE) followed in a tertiary fetal–maternal center. Data were collected from clinical charts between January 1993 and December 2007, with a total of 136 pregnancies (107 patients). Mean maternal age was 29 years, with the vast majority of patients being Caucasian. Most patients were in remission 6 months prior to pregnancy (93%) and the most frequently affected organs were the skin and joints. Renal lupus accounted for 14% of all cases. Twenty-nine percent of patients were positive for at least one antiphospholid antibody (aPL) and nearly 50% had positive SSa/SSb antibodies. All patients with positive aPL received low-dosage aspirin and low molecular- weight heparin (LMWH). There were no pregnancy complications in more than 50% of cases and hypertensive disease and intrauterine growth restriction were the most common adverse events. There were 125 live births, one neonatal death, eight miscarriages, and three medical terminations of pregnancy. Preterm delivery occurred in 25% of pregnancies. Our results are probably the conjoined result of a multidisciplinary approach together with a systematic management of SLE pregnancies, with most patients keeping their prior SLE medication combined with low-dosage aspirin and LMWH in the presence of aPL.

Drug Provocation Tests to Betalactam Antibiotics: Experience in a Paediatric Setting

Background: Few studies have been performed in children withs uspected betalactam allergy.We aimed to assess the role of the drug provocation test(DPT)with betalactams in a paediatric setting and to study the association between allergy to betalactam antibiotics and other allergic diseases. Methods:We included all the patients under 15 years old who were consecutively referred to the Immunoallergy Department, Dona Estefânia Hospital,Portugal(January 2002 to April 2008)for a compatible history of allergic reaction to betalactam. All were submitted to a DPT.Children were proposed to performs kintests(ST)to betalactam antibiotics followed by DPT. If they decline ST,a DPT with the culprit drug was performed. Results: We studied 161 children,60%were boys,with a median age of 5years old at the time of the DPT.Thirty-three patients(20.5%)had an immediate reaction and 33(20.5%)a non-immediate reaction. These verity of there porte dreactions was low in most cases. Skin tests to betalactams were performed in 47 children and were positive in 8.DPT was positive inonlyone(3.4%)of the patients skin tested and in 11(13.4%)of those not skin tested. These verity of the DPT reaction was low.Asthma and food allergy were associated with a positive DPT in the later group. Conclusions: DPT seems a safe procedure even in the absence of ST in non-severe cases. This could be a practical optionin infants and pre-school children,where ST are painful and difficult to perform.Additional caution should be taken in children with asthma and food allergy.

Primary Disorders of Neurotransmitter Metabolism: Experience of a Tertiary Center

Neurotransmitter diseases are a group of inherited disorders attributable to a disturbance of neurotransmitter metabolism. Biogenic amines are neurotransmitters with multiple roles including psychomotor function, hormone secretion, cardiovascular, respiratory and gastrointestinal control, sleep mechanisms, body temperature and pain. Given the multiple functions of monoamines, disorders of their metabolism comprise a wide spectrum of manifestations, with motor dysfunction being the most prominent clinical feature. Methods: Case review of 12 patients from 4 families, with primary disorders of biogenic amine metabolism. Results: Aromatic L-amino acid decarboxylase deficiency (4 patients from 2 families), and GTP-cyclohydrolase (8 patients from 2 families) were the two diseases identified. Age at first symptoms varied between 2 months and 6 years. Developmental delay was present in all cases except 2 patients with GTP cyclohydrolase deficiency. The combination of axial hypotonia and limb dystonia was also frequent. Children with aromatic L-amino acid decarboxylase deficiency exhibited temperature instability, oculogyric crisis and disturbances of sleep. The index case of one family with GTP cyclohydrolase deficiency presented with Parkinsonism (bradykinesia, rigidity and hypomimia). Analysis of neurotransmitters and their metabolites in CSF was crucial for the identification of index cases. Response to therapy was variable but in general unsatisfactory except in a family with GTP cyclohydrolase deficiency. Conclusions: These disorders should be considered in the differential diagnosis of paediatric neurodegenerative diseases, in order to allow an adequate therapeutic trial that can favor prognosis.

Epidemiology of neglected tropical diseases in transplant recipients: review of the literature and experience of a Brazilian HSCT center

The rising success rate of solid organ (SOT) and haematopoietic stem cell transplantation (HSCT) and modern immunosuppression make transplants the first therapeutic option for many diseases affecting a considerable number of people worldwide. Consequently, developing countries have also grown their transplant programs and have started to face the impact of neglected tropical diseases (NTDs) in transplant recipients. We reviewed the literature data on the epidemiology of NTDs with greatest disease burden, which have affected transplant recipients in developing countries or may represent a threat to transplant recipients living in other regions. Tuberculosis, Leprosy, Chagas disease, Malaria, Leishmaniasis, Dengue, Yellow fever and Measles are the topics included in this review. In addition, we retrospectively revised the experience concerning the management of NTDs at the HSCT program of Amaral Carvalho Foundation, a public transplant program of the state of São Paulo, Brazil.

Ten years experience with Jorge Lobo's disease in the state of Acre, Amazon region, Brazil

Jorge Lobo's disease is a cutaneous and subcutaneous mycosis that affects patients in the Amazon region. The number of patients is relatively small, but the real situation of the disease as public health problem is not known, because Jorge Lobo's disease is not a notifiable disease. This study aims to report the clinical evolution in patients affected and to determine the prevalence and areas of occurrence of the disease. A retrospective study was carried out based on the analysis of the clinical records, which included a collection of photographs of patients in the Department of Sanitary Dermatology, in Rio Branco, and patients seen in the interior of the state. In a decade, in Rio Branco, 249 cases of the disease were reported, 30 were females and 219 males. Of these patients, 153 had localized lesions, 94 of them were on one ear, 55 had multifocal lesions and 41 had disseminated lesions. The average time between the onset of symptoms and diagnosis was 19 years. The average age at the time of diagnosis was 53 years, and ages ranged from 14 to 96 years.

Cutaneous sporotrichosis treatment with potassium iodide: a 24 year experience in São Paulo state, Brazil

BACKGROUND: Sporotrichosis is a subacute or chronic disease caused by a dimorphic fungus, Sporothrix schenckii. The first and most traditional treatment is potassium iodide in satured solution (SSKI) used by DE BEURMANN in 1907. For its effectiveness, it is still used for cutaneous sporotrichosis. OBJECTIVE: To evaluate the treatment of cutaneous sporotrichosis with SSKI in relation to clinical cure, side effects, length of treatment and reactivation. METHODS: We conducted a retrospective analysis of medical records over a 24-year period (1981-2005). Patients of all ages who were treated in the hospital´s division of dermatology were included in the study providing that they had a positive culture of S. schenckii. Satured solution of potassium iodide (3 to 6g per day) was the treatment prescribed. For children, half of the dose was prescribed. RESULTS: The lymphocutaneous disease was prevalent, the cure rate was 94.7%, side effects were described in 5.5% of the cases, mean length of treatment was 3.5 months and possible reactivation was observed in 11.1%. CONCLUSION: SSKI is an effective drug, with many side effects, but with low frequency. Resolution was for maximum six months of treatment. SSKI has been found to be a very effective drug in this retrospective study of culture-proven cases of cutaneous and lymphocutaneous sporotrichosis. It should be used as first drug of choice especially in resource-limited settings.

Potencialidades de uma abordagem com Interdisciplinaridade entre matemática e estudo do meio e centrada no ambiente

O presente estudo partiu do problema “Como promover aprendizagens da Matemática e do Estudo do Meio numa perspetiva interdisciplinar, explorando o mundo real?”. Neste sentido, tem como objetivos: selecionar recursos e atividades que se revelem motivadoras para os alunos; demonstrar a relevância da inter-relação de diferentes conceitos e a importância da sua ligação com as vivências dos alunos; ativar o envolvimento dos alunos para a aprendizagem da Matemática através do Estudo do meio e de situações do mundo real; estimular a perceção do aluno da presença da Matemática nos conteúdos de Estudo do Meio; fomentar a compreensão da relação dos conteúdos de Matemática e estudo do Meio. Com este propósito formularam-se as seguintes questões: (1) Que tipo de atividades se poderão proporcionar de forma a motivar os alunos para os conteúdos do Estudo do Meio e da Matemática? (2) De que forma a exploração das situações/conceções do quotidiano poderá promover o envolvimento dos alunos na aprendizagem da Matemática e do Estudo do Meio? (3) De que forma o Ensino Experimental das Ciências numa perspetiva interdisciplinar pode contribuir para desenvolver tanto as competências conceptuais (fatores do ambiente: temperatura e humidade/OTD/números racionais), como capacidades de pensamento crítico e tomada de decisão inerente? Tendo em vista os objetivos do estudo, desenvolveram-se, com uma turma do 2º ano de escolaridade, quatro situações formativas, que envolveram as disciplinas de Matemática e Estudo do Meio. O domínio de conteúdos preponderante na área de Estudo do Meio foi À descoberta do Ambiente Natural, enquanto na Matemática os domínios predominantes foram Organização e tratamento de dados e Números e operações. Foram realizadas diversas atividades experimentais, onde os alunos tiveram um papel ativo na construção dos seus conhecimentos. A investigação segue uma metodologia qualitativa, centrando-se num estudo de caso, onde se caracteriza uma experiência interdisciplinar que envolveu as disciplinas de Matemática e Estudo do Meio. Os dados foram recolhidos pela professora investigadora através de gravações de vídeo e áudio, fotografias, trabalhos dos alunos e de registos da professora investigadora. Os resultados demonstraram como os alunos mobilizaram e apropriaram os conteúdos de Matemática e Estudo do Meio. Os dados, através da análise de conteúdo, parecem iv sugerir que houve uma evolução no desempenho dos alunos a vários níveis, nomeadamente: no trabalho cooperativo, no envolvimento da tarefa, nas interações estabelecidas e na motivação para a aprendizagem da Matemática e Estudo do Meio.

Relatório de Estágio de Qualificação Profissional (Educação Pré-Escolar)

Relatório EPE - Relatório de Estágio em Educação Pré-Escolar: Para aprender a ensinar é necessário passar por diversas etapas, assim sendo, o presente relatório é o culminar de um primeiro momento de estágio, do primeiro contacto com a profissão com todas as dinâmicas e especificidades de que dela fazem parte. Pretendendo-se ser um educador reflexivo e investigador, em que o desejo de saber o porquê daquilo que observa, o leva a descobrir novas coisas e a refletir sempre sobre elas, é fulcral que se conheçam alguns pressupostos teóricos que sirvam de suporte à prática a realizar. Desta forma, é feita uma contextualização teórica em que se aborda o papel do Educador de Infância, agente tão importante na educação das crianças, uma vez que educa os cidadãos de amanhã. Ao mesmo tempo, são ainda abordados alguns tópicos pertinentes relativamente à educação de infância, como a organização do ambiente educativo, a intencionalidade educativa, assim como modelos e perspetivas teóricas que fundamentam toda a prática desenvolvida. Com a realização deste relatório, inicia-se a construção do profissional reflexivo e investigador. Neste sentido, reflete-se acerca de toda ação pedagógica desenvolvida, assim como dos aspetos que se devem melhorar no futuro e que objetivos anteriormente definidos foram já alcançados. Na certeza de que o período de prática pedagógica referenciada foi o primeiro de muitos momentos de autodescoberta e realização pessoal, espera-se que o mesmo seja capaz de transparecer toda a experiência e enriquecimento para quem o lê. Relatório EPE - Relatório de Estágio em Educação Pré-Escolar:In order to learn how to teach, one needs to follow several stages, and on that path, the present report is the final step of a first stage in the trainee program, one that marked the first contact with the teaching profession and with all its dynamics and specifics. The aim is to be a reflexive educator and researcher, one that desires to understand the reason and motive of its object, one that is led to the discovery of new things and to a reflection on them. On that line, it is important to know some theoretical concepts to support the practice. With that goal in mind, this paper is a theoretical contextualization relating to the role of the infant teacher, a very important one, as it concerns the education of future citizens. At the same time, some relevant topics are addressed in what concerns the work of the infant teacher, such as the arrangement of the educational environment, the educational intentionality, and also theoretical models and perspectives, fundamental to the developed work. With this report begins the construction of the reflexive professional and researcher. In doing so, it is a reflection on several issues, namely, on all the pedagogical actions developed throughout the stage, on the features to improve in the future, and what previously defined goals were already achieved. Without a doubt, this stage of educational action is the first of several moments of self-discovery and personal achievement. Hopefully, this report will be able to reflect all the experience and to be an enriching experience to the reader.

Research and development expenditure in the business sector as indicator of knowledge economy: the Portuguese experience

The objective of the paper is to help to understand recent changes in the structure of R&D activities, by analyzing data on the expenditure of the business sector in research and development (R&D). The results are framed in an international context, through comparison with indicators from the most developed countries, divided by technological intensity and economic activity. The study reveals that the indicators of Portuguese R&D expenditure in the business sector are closely linked both to fiscal policy and to high foreign direct investment in knowledge-intensive industries. It also links these indicators to phenomena such as the abundance of skilled labor in pharmaceutical industries and the government intervention in some sectors of the economy (namely health and rail transportation).

Safety and Effectiveness of the Genous™ Endothelial Progenitor Cell-Capture Stent in the First Year Following ST-Elevation Acute Myocardial Infarction: A Single Center Experience and Review of the Literature

PURPOSE: The Genous™ stent (GS) is designed to accelerate endothelization, which is potentially useful in the pro-thrombotic environment of ST-elevation acute myocardial infarction (STEMI). We aimed to evaluate the safety and effectiveness of the GS in the first year following primary percutaneous coronary intervention (PCI) and to compare our results with the few previously published studies. METHODS AND MATERIALS: All patients admitted to a single center due to STEMI that underwent primary PCI using exclusively GS, between May 2006 and January 2012, were enrolled. The primary study endpoints were major adverse cardiac events (MACEs), defined as the composite of cardiac death, acute myocardial infarction and target vessel revascularization, at one and 12months. RESULTS: In the cohort of 109 patients (73.4% male, 59 ±12years), 24.8% were diabetic. PCI was performed in 116 lesions with angiographic success in 99.1%, using 148 GS with median diameter of 3.00mm (2.50-4.00) and median length of 15mm (9-33). Cumulative MACEs were 2.8% at one month and 6.4% at 12months. Three stent thromboses (2.8%), all subacute, and one stent restenosis (0.9%) occurred. These accounted for the four target vessel revascularizations (3.7%). At 12months, 33.9% of patients were not on dual antiplatelet therapy. CONCLUSIONS: GS was safe and effective in the first year following primary PCI in STEMI, with an apparently safer profile comparing with the previously published data. SUMMARY: We report the safety and effectiveness of the Genous™ stent (GS) in the first year following primary percutaneous coronary intervention in ST-elevation acute myocardial infarction. A comprehensive review of the few studies that have been published on this subject was included and some suggest a less safe profile of the GS. Our results and the critical review included may add information and reinforce the safety and effectiveness of the GS in ST-elevation in acute myocardial infarction.

Alcohol Septal Ablation in Obstructive Hypertrophic Cardiomyopathy: Four Years of Experience at a Reference Center

INTRODUCTION: We describe our center's initial experience with alcohol septal ablation (ASA) for the treatment of obstructive hypertrophic cardiomyopathy. The procedure, its indications, results and clinical outcomes will be addressed, as will its current position compared to surgical myectomy. OBJECTIVE: To assess the results of ASA in all patients treated in the first four years of activity at our center. METHODS: We retrospectively studied all consecutive and unselected patients treated by ASA between January 2009 and February 2013. RESULTS: In the first four years of experience 40 patients were treated in our center. In three patients (7.5%) the intervention was repeated. Procedural success was 84%. Minor complications occurred in 7.5%. Two patients received a permanent pacemaker for atrioventricular block (6% of those without previous pacemaker). The major complication rate was 5%. There were no in-hospital deaths; during clinical follow-up (22 ± 14 months) cardiovascular mortality was 2.5% and overall mortality was 5%. DISCUSSION AND CONCLUSION: The results presented reflect the initial experience of our center with ASA. The success rate was high and in line with published results, but with room to improve with better patient selection. ASA was shown to be safe, with a low complication rate and no procedure-related mortality. Our experience confirms ASA as a percutaneous alternative to myectomy for the treatment of symptomatic patients with obstructive hypertrophic cardiomyopathy refractory to medical treatment.