Case for Diagnosis

Blastic plasmacytoid dendritic cell tumor is a rare, highly aggressive systemic neoplasm for which effective therapies have not yet been established. We describe a 73-year-old man with multiple nodules and patches emerging on the trunk and limbs. Lesional skin biopsy revealed a plasmacytoid dendritic cell tumor with dense dermal infiltrate of tumor cells with blastoid features. No apparent systemic involvement was identified in the initial stage. The patient was treated with prednisone daily, with notorious improvement of the skin lesions, although no complete remission was obtained. During the six-month follow-up period, no disease progression was documented, but fatal systemic progression occurred after that period of time.

Focal Malignant Liver Lesions: Diagnosis by Dynamic Incremental CT, Early Phase

The purpose of our study was to evaluate the accuracy of dynamic incremental bolus-enhanced conventional CT (DICT) with intravenous contrast administration, early phase, in the diagnosis of malignancy of focal liver lesions. A total of 122 lesions were selected in 74 patients considering the following criteria: lesion diameter 10 mm or more, number of lesions less than six per study, except in multiple angiomatosis and the existence of a valid criteria of definitive diagnosis. Lesions were categorized into seven levels of diagnostic confidence of malignancy compared with the definitive diagnosis for acquisition of a receiver-operator-characteristic (ROC) curve analysis and to determine the sensitivity and specificity of the technique. Forty-six and 70 lesions were correctly diagnosed as malignant and benign, respectively; there were 2 false-positive and 4 false-negative diagnoses of malignancy and the sensitivity and specificity obtained were 92 and 97%. The DICT early phase was confirmed as a highly accurate method in the characterization and diagnosis of malignancy of focal liver lesions, requiring an optimal technical performance and judicious analysis of existing semiological data.

Como responder à heterogeneidade dos alunos, de modo a proporcionar o sucesso de cada um?

O sucesso educativo dos alunos é como facilmente se compreenderá, a essência do ato educativo nas suas múltiplas dimensões e formas. O percurso para a obtenção desse sucesso é variado e representa muitas vezes um enorme investimento das organizações educativas nomeadamente na gestão dos seus recursos humanos. Por outro lado, o modelo organizativo instituído pode ser o fator preponderante para a obtenção do sucesso procurado sobretudo quando ele é promotor de um clima harmonioso e motivador para o ato educativo. O projeto apresentado neste estudo estruturou-se numa nova construção dos grupos de trabalho, em que o conceito tradicional de turma foi substituído por grupos, constituídos por alunos cujo grau de conhecimento e competências no âmbito das disciplinas de Português e Matemática, fosse o mais aproximado possível não perdendo de vista o princípio de heterogeneidade do grupo mesmo que relativa ou diminuída, tão importante para promover a diversidade. Este sistema organizativo rompeu com o conceito tradicional da turma o que implicou uma alteração não só na organização de horários escolares dos alunos e docentes mas também obrigou estruturação e implementação de um processo de mobilidades dos alunos entre grupos em função do seu grau de aprendizagens ou de dificuldades na sua obtenção. Este projeto foi ainda promotor de uma cultura de trabalho colaborativo e partilha entre docentes de cada grupo disciplinar promovendo a sistematização de interações entre pares quer na preparação das atividades letivas quer na discussão e resolução dos constrangimentos que o desenvolvimento do projeto evidenciou.

Blastic Plasmacytoid Dendritic Cell Neoplasm

Blastic plasmacytoid dendritic cell neoplasm is a rare and aggressive hematodermic neoplasia with frequent cutaneous involvement and leukemic dissemination. We report the case of a 76-year-old man with a 2 month history of violaceous nodules and a tumor with stony consistency, located on the head, and mandibular, cervical and supraclavicular lymphadenopathies. Multiple thoracic and abdominal adenopathies were identified on computerized tomography. Flow cytometry analysis of the skin, lymph node and bone marrow biopsies demonstrated the presence of plasmocytoid dendritic cell neoplastic precursor cells (CD4+, CD45+, CD56+ and CD123+ phenotype). After initial clinical and laboratorial complete remission with chemotherapy, the patient died due to relapse of the disease associated with the appearance of a cervical mass with medullary compromise.

INFECTION BY Rickettsia felis IN OPOSSUMS (Didelphis sp.) FROM YUCATAN, MEXICO

Rickettsia felis is an emergent pathogen and the causative agent of a typhus-like rickettsiosis in the Americas. Its transmission cycle involves fleas as biological vectors (mainly Ctenocephalides felis) and multiple domestic and synanthropic mammal hosts. Nonetheless, the role of mammals in the cycle of R. felis is not well understood and many efforts are ongoing in different countries of America to clarify it. The present study describes for the first time in Mexico the infection of two species of opossum (Didelphis virginiana and D. marsupialis) by R. felis. A diagnosis was carried out from blood samples by molecular methods through the gltAand 17 kDa genes and sequence determination. Eighty-seven opossum samples were analyzed and 28 were found to be infected (32.1%) from five out of the six studied localities of Yucatan. These findings enable recognition of the potential epidemiological implications for public health of the presence of infected synanthropic Didelphis in households.

Mieloma Múltiplo e Amiloidose AL

O mieloma múltiplo e a amiloidose de cadeias leves (AL) de imunoglobulina são doenças proliferativas plasmocitárias incuráveis que raramente coexistem. Cerca de 10 a 15% dos doentes com mieloma múltiplo desenvolvem amiloidose AL clinicamente sintomática, sendo a situação inversa muito rara (0,4%). Apresenta-se um caso não previamente diagnosticado de mieloma múltiplo não secretor, isto é, sem identificação de paraproteína na imunoelectroforese ou imunofixação sérica ou urinária, numa doente internada por icterícia pruriginosa e volumosa hepatomegália. A biópsia hepática confirmou o diagnóstico de amiloidose e o doseamento sérico das cadeias leves livres das imunoglobulinas revelou hiperprodução de cadeias leves kappa (k). Os autores abordam as particularidades clínicas e de investigação destas duas entidades em que o diagnóstico precoce e a terapêutica atempada influenciam o prognóstico.

ClearPhoto - augmented photography

The widespread use of mobile devices has made known to the general public new areas that were hitherto confined to specialized devices. In general, the smartphone came to give all users the ability to execute multiple tasks, and among them, take photographs using the integrated cameras. Although these devices are continuously receiving improved cameras, their manufacturers do not take advantage of their full potential, since the operating systems normally offer simple APIs and applications for shooting. Therefore, taking advantage of this environment for mobile devices, we find ourselves in the best scenario to develop applications that help the user obtaining a good result when shooting. In an attempt to provide a set of techniques and tools more applied to the task, this dissertation presents, as a contribution, a set of tools for mobile devices that provides information in real-time on the composition of the scene before capturing an image. Thus, the proposed solution gives support to a user while capturing a scene with a mobile device. The user will be able to receive multiple suggestions on the composition of the scene, which will be based on rules of photography or other useful tools for photographers. The tools include horizon detection and graphical visualization of the color palette presented on the scenario being photographed. These tools were evaluated regarding the mobile device implementation and how users assess their usefulness.

Of Structural Denial: A Narratological Study of the Structural Disintegration of the Novel Form

If an opening to the argument of this dissertation is of imperative necessity, one might tentatively begin with Herbert Quain, born in Roscommon, Ireland, author of the novels The God of the Labyrinth (1933) and April March (1936), the short-story collection Statements (1939), and the play The Secret Mirror (undated). To a certain extent, this idiosyncratic Irish author, who hailed from the ancient province of Connacht, may be regarded as a forerunner of the type of novels which will be considered in this dissertation. Quain was, after all, the unconscious creator of one of the first structurally disintegrated novels in the history of western literature, April March. His first novel, The God of the Labyrinth, also exhibits elements which are characteristic of structurally disintegrated fiction, for it provides the reader with two possible solutions to a mysterious crime. As a matter of fact, one might suggest that Quain’s debut novel offers the reader the possibility to ignore the solution to the crime and carry on living his or her readerly life, turning a blind eye to the novel itself. It may hence be argued that Quain’s first novel is in fact a compound of three different novels. It is self-evident that the structure of Quain’s oeuvre is of an experimental nature, combining geometrical precision with authorial innovation, and one finds in it a higher consideration for formal defiance than for the text itself. In other words, the means of expression are the concern of the author and not, interestingly, the textual content. April March, for example, is a novel which regresses back into itself, its first chapter focussing on an evening which is preceded by three possible evenings which, in turn, are each preceded by three other, dissimilar, possible evenings. It is a novel of backward-movement, and it is due to this process of branching regression that April March contains within itself at least nine possible novels. Structure, therefore, paradoxically controls the text, for it allows the text to expand or contract under its formal limitations. In other words, the formal aspects of the novel, usually associated with the restrictive device of a superior design, contribute to a liberation of the novel’s discourse. It is paradoxical only in the sense that the idea of structure necessarily entails the fixation of a narrative skeleton that determines how plot and discourse interact, something which Quain flouts for the purposes of innovation. In this sense, April March’s convoluted structure allows for multiple readings and interpretations of the same text, consciously germinating narratives within itself, producing different texts from a single, unique source. Thus, text and means of expression are bonded by a structural design that, rather than limiting, liberates the text of the novel.

Learning new tricks: Corticostriatal dynamics during novel skill learning

Learning novel actions and skills is a prevalent ability across multiple species and a critical feature for survival and competence in a constantly changing world. Novel actions are generated and learned through a process of trial and error, where an animal explores the environment around itself, generates multiple patterns of behavior and selects the ones that increase the likelihood of positive outcomes. Proper adaptation and execution of the selected behavior requires the coordination of several biomechanical features by the animal. Cortico-basal ganglia circuits and loops are critically involved in the acquisition, learning and consolidation of motor skills.(...)

Doenças raras na Ilha de S. Miguel : caracterização epidemiológica

RESUMO:O conceito de doenças raras como entidade própria começou a ser divulgado na comunidade médica no início deste século. A perspectiva de congregar múltiplas patologias, com características diferentes, valorizando a baixa frequência com que ocorrem na população interessou a comunidade científica, famílias, indústria e serviços de saúde. Esperava-se encontrar estratégias para melhorar a qualidade dos cuidados de saúde prestados a estes doentes. Uma vez que a informação científica sobre doenças raras está dispersa por diversas fontes o primeiro grande desafio foi sistematizar de forma a obter o “estado da arte”. A investigação que decorreu entre 2001 e 2010 teve como objectivo principal a caracterização dos doentes e das doenças raras numa população com características restritas mas não fechada como é o caso da ilha de S. Miguel nos Açores. Foram identificados 467 doentes a partir de várias fontes e monitorizado o nascimento de recém-nascidos com doença rara durante 10 anos. A prevalência das doenças raras encontrada na ilha de S. Miguel foi de 0,34% e a inerente à definição de doença rara foi de 6 % a 8 % da população na União Europeia. A diferença encontrada poderá decorrer de se ter sobrestimado o verdadeiro valor da prevalência das doenças raras na União Europeia. A incidência de doenças raras determinada na amostra foi de 0,1% e a taxa de mortalidade por causa específica foi de 0,14‰. O diagnóstico foi confirmado por técnicas laboratoriais de citogenética ou genética molecular em 43% dos doentes da amostra. Não foi identificado nenhum agregado populacional com doença rara para além do já conhecido para a DMJ. A criação de uma metodologia de estudo implicou a construção de um registo de doentes. Para tal foi utilizado o conhecimento adquirido anteriormente sobre uma doença rara que serviu de paradigma: a doença de Machado-Joseph. Na sequência dos resultados obtidos foi considerado útil a introdução de variáveis como a figura do cuidador, o cônjuge, o número de filhos do casal, a data da primeira consulta de Genética, o tempo decorrido entre o início dos sintomas e o acesso à consulta de Genética e entre esta actividade e dispor do diagnóstico para melhor compreender o contexto de vida destes doentes na perspectiva de poderem vir a ser incorporadas como indicadores. ----------- ABSTRACT: The concept of rare diseases as a condition began to be disclosed in the medical community at the beginning of this century.The prospect of bringing together multiple pathologies, with different features, enhancing the low frequency with which they occur in the population interested the scientific community, families, industry and health care services. The aim was to find strategies to improve the quality of care provided to these patients. Given that the scientific information on rare diseases is spread out across several sources the first major challenge was to systematize in order to get the "state of the art". The research took place between 2001 and 2010 and had as its main objective the characterization of patients and rare diseases in a population with specific features, but not confined, like in the case of the São Miguel Island in Azores. During 10 years were identified 467 patients from multiple sources and were observed the newborns with rare diseases. Prevalence of rare diseases found in the São Miguel Island was 0,34% compared to the 6% to 8% by definition of rare disease in the population in European Union. This discrepancy may be explained by a likely frequency of overrated rare diseases in European Union. The incidence of rare diseases in the sample was 0,1% and the specific mortality rate was 0,14 ‰. This diagnosis was confirmed by cytogenetic or molecular genetics analysis in 43% of patients in the sample. No population cluster was identified with rare disease besides the already known for Machado-Joseph Disease. The methodology for the study involved the construction of a database of patients. For such purpose it was used previously acquired knowledge on a rare disease paradigm: the Machado-Joseph disease. It was useful to introduce the following variables to properly establish the results: caregiver, spouse, number of children, date of first Genetics appointment, elapsed time between onset of symptoms and access to first appointment as well as this and the final diagnosis to better understand the context of life of these patients in order to incorporate them as rates.

Construção de uma responsabilidade europeia além-fronteiras

The suppression of internal border controls has led the European Union to establish a mechanism for determining the Member State responsible for examining each asylum application, with the main intention of deterring asylum seekers from lodging multiple applications and guaranteeing that it will be assessed by one of the States – the Dublin System. Even though it holds on a variety of criteria, the most commonly used is the country of first entrance in the EU. The growing migrating flows coming mainly from Northern Africa have thus resulted in an incommensurable burden over the border countries. Gradually, countries like Greece, Bulgaria and Italy have lost capability of providing adequate relief to all asylum seekers and the records of fundamental rights violations related to the provision of housing and basic needs or inhuman detention conditions started piling up. To prevent asylum seekers who had already displaced themselves to other Member States from being transferred back to countries where their human dignity is questionable, the European Court of Human Rights and the Court of Justice have developed a solid jurisprudence determining that when there is a risk of serious breach of fundamental rights all transfers to that country must halt, especially when it is identified with systemic deficiencies in the asylum system and procedures. This reflexion will go through the jurisprudence that influenced very recent legislative amendments, in order to identify which elements form part of the obligation not to transfer under the Dublin System. At last, we will critically analyze the new rising obligation, that has clearly proven insufficient in light of the international fundamental rights framework that the Member States and the EU are bound to respect, proposing substantial amendments with a view to reach a future marked by high solidarity and global responsibility from the European Union.

Finding the hidden influencers

This thesis is a first step in the search for the characteristics of funders, and the underlying motivation that drives them to participate in crowdfunding. The purpose of the study is to identify demographics and psychographics that influence a funder’s willingness to financially support a crowdfunding project (WFS). Crowdfunding, crowdsourcing and donation literature are combined to create a conceptual model in which age, gender, altruism and income, together with several control variables, are expected to have an influence on a funder’s WFS. Primary data collection was conducted using a survey, and a dataset of 175 potential crowdfunders was created. The data is analysed using a multiple regression and provided several interesting results. First of all, age and gender have a significant effect on WFS, males and young adults until the age of 30 have a higher intention to give money to crowdfunding projects. Second, altruism is significantly positively related to WFS, meaning that the funders do not just care about the potential rewards they could receive, but also about the benefits that they create for the entrepreneur and the people affected by the crowdfunding project. Third, the moderation effect of income was found to be insignificant in this model. It shows that income does not affect the strength of the relationship between the age, gender and altruism, and WFS. This study provides important theoretical contributions by, to the best of my knowledge, being the first study to quantitatively investigate the characteristics of funders and using the funder as the unit of analysis. Moreover, the study provides important insights for entrepreneurs who wish to target the crowd better in order to attract and retain more funders, thereby increasing the chance of success of their project.

Mais além de quem escreve: “Eu também posso ser Ilustrador”: desenvolvendo múltiplas linguagens num projeto curricular integrado

Relatório de estágio de mestrado em Educação Pré-Escolar

Abundance of two Dendrocincla woodcreepers (aves: Dendrocolaptidae) in relation to forest structure in Central Amazonia

Few studies have been conducted to verify how the structure of the forest affects the occurence and abundance of neotropical birds. Our research was undertaken between January 2002 and July 2004 at the Reserva Ducke, near Manaus (02º55',03º01'S; 59º53',59º59'W) in central Amazonia, to verify how the forest structure affects the occurrence and abundance of two bird species: the Plain-brown Woodcreeper Dendrocincla fuliginosa and the White-chinned Woodcreeper Dendrocincla merula. Bird species occurrence was recorded using lines of 20 mist-nets (one sample unit), along 51 1-km transects distributed along 9 pararel 8 km trails covering an area of 6400 ha. Along these transects, we placed 50 x 50m plots where we recorded forest structure components (tree abundance, canopy openness, leaf litter, standing dead trees, logs, proximity to streams, and altitude). We then related these variables to bird occurence and abundance using multiple logistic and multiple linear regression models, respectively. We found that D. fuliginosa frequently used plateau areas; being more abundant in areas with more trees. On the other hand, D. merula occurred more frequently and was more abundant in areas with low tree abundance. Our results suggest that although both species overlap in the reserve (both were recorded in at least 68% of the sampled sites), they differ in the way they use the forest microhabitats. Therefore, local variation in the forest structure may contribute to the coexistence of congeneric species and may help to maintain local alpha diversity.

From the periphery to the brain: Lipocalin-2, a friend or foe?

Lipocalin-2 (LCN2) is an acute-phase protein that, by binding to iron-loaded siderophores, acts as a potent bacteriostatic agent in the iron-depletion strategy of the immune system to control pathogens. The recent identification of a mammalian siderophore also suggests a physiological role for LCN2 in iron homeostasis, specifically in iron delivery to cells via a transferrin-independent mechanism. LCN2 participates, as well, in a variety of cellular processes, including cell proliferation, cell differentiation and apoptosis, and has been mostly found up-regulated in various tissues and under inflammatory states, being its expression regulated by several inducers. In the central nervous system less is known about the processes involving LCN2, namely by which cells it is produced/secreted, and its impact on cell proliferation and death, or in neuronal plasticity and behaviour. Importantly, LCN2 recently emerged as a potential clinical biomarker in multiple sclerosis and in ageing-related cognitive decline. Still, there are conflicting views on the role of LCN2 in pathophysiological processes, with some studies pointing to its neurodeleterious effects, while others indicate neuroprotection. Herein, these various perspectives are reviewed and a comprehensive and cohesive view of the general function of LCN2, particularly in the brain, is provided.