Clinical and radiological aspects in Melnick-Needles syndrome

Melnick-Needles syndrome is an X-linked dominant bone dysplasia, lethal in males, characterized by a typical facies and characteristic radiological findings: including sclerosis of skull base and mastoids, S-shaped appearance of tibia; cortical irregularities with a ribbon appearance of the ribs. About 48 well-documented cases have been reported, most of them were sporadic. Parental transmission has been published in only 11 kindreds. We are presenting the first Brazilian family with mother-daughter transmission.The proposita presented the typical clinical and radiological features with characteristic facies, severe thoracic cage restriction and pulmonary hypertension. Her mother was more mildly affected.

Radiographic aspects and angioarchitectural arrangements in corrosion casts of the blood supply to the human sternocleidomastoid muscle by the sternocleidomastoid branch of the occipital artery

The contribution of the sternocleidomastoid branch of the occipital artery (superior arterial pedicle - SAP) to the irrigation of the sternocleidomastoid muscle (SCM) was evaluated in fresh human cadavers by injecting radiological dye and a resin for microvasculature corrosion casts. From its insertion in the mastoid process of the temporal bone, the SCM was divided into superior, medium, and inferior thirds. In most of the SCM, The SAP are formed by two longitudinal parallel branches. In all specimens, the radiological dye injected into the SAP reached or trespassed the middle part of the studied SCM. The SAP was poorly distributed in the lowermost region of the inferior third of the SCM, suggesting the contribution of other arteries or pedicles. The corrosion casts of the microvasculature showed a profuse network of microscopic vessels in those levels where the SAP was detected.

Measuring male infertility: epidemiological aspects

Evidence suggests that human semen quality may have been deteriorating in recent years. Most of the evidence is retrospective, based on analysis of data sets collected for other purposes. Measures of male infertility are needed if we want to monitor the biological capacity for males to reproduce over time or between different populations. We also need these measures in analytical epidemiology if we want to identify risk indicators, risk factors, or even causes of an impaired male fecundity-that is, the male component in the biological ability to reproduce. The most direct evaluation of fecundity is to measure the time it takes to conceive. Since the time of conception may be missed in the case of an early abortion, time to get pregnant is often measured as the time it takes to obtain a conception that survives until a clinically recognized pregnancy or even a pregnancy that ends with a live born child occurs. A prolonged time required to produce pregnancy may therefore be due to a failure to conceive or a failure to maintain a pregnancy until clinical recognition. Studies that focus on quantitative changes in fecundity (that does not cause sterility) should in principle be possible in a pregnancy sample. The most important limitation in fertility studies is that the design requires equal persistency in trying to become pregnant and rather similar fertility desires and family planning methods in the groups to be compared. This design is probably achievable in exposure studies that make comparisons with reasonable comparable groups concerning social conditions and use of contraceptive methods.

Ethical aspects in the management of the terminally ill patient in the pediatric intensive care unit

OBJECTIVE: To identify the prevalence of management plans and decision-making processes for terminal care patients in pediatric intensive care units. METHODOLOGY: Evidence-based medicine was done by a systematic review using an electronic data base (LILACS, 1982 through 2000) and (MEDLINE, 1966 through 2000). The key words used are listed and age limits (0 to 18 years) were used. RESULTS: One hundred and eighty two articles were found and after selection according to the exclusion/inclusion criteria and objectives 17 relevant papers were identified. The most common decisions found were do-not-resuscitation orders and withdrawal or withholding life support care. The justifications for these were "imminent death" and "unsatisfatory quality of life". CONCLUSION: Care management was based on ethical principles aiming at improving benefits, avoiding harm, and when possible, respecting the autonomy of the terminally ill patient.

Dentro e fora de si: da fotografia como objecto cultural

Este artigo propõe um alargamento na abordagem normalmente aplicada à fotografia em Portugal, reclamando um espaço de abertura disciplinar e metodológica que permita, por um lado, deslocar o olhar do investigador de uma história feita de centros e de momentos marcantes e, por outro, reflectir sobre as várias dimensões discursivas e socioculturais em que a imagem fotográfica existe e dialoga com os seus públicos. Na linha da abertura fundadora que os estudos culturais provocaram na estrutura das Humanidades interessará, do mesmo modo, fomentar uma investigação baseada numa consciente focalização no objecto de estudo que seja capaz de articular diversos contributos teóricos e disciplinares no campo unificado dos estudos fotográficos. Partindo não apenas da profunda enunciação que Jean‑Pierre Esquenazi traça da noção de instituição, mas também da reformulação do termo estudos culturais que Mieke Bal propõe com o conceito de análise cultural, este artigo procurará sobretudo caracterizar e debater a pertinência de um campo de estudo para a fotografia, que se pretenda transnacional, interdisciplinar e metodologicamente flexível, e que permita abarcar criticamente as várias dimensões culturais que a imagem fotográfica sempre cumpre.

Património Cultural Imaterial, Festa e Comunidade

A presente contribuição toma como ponto de partida a centralidade do conceito de comunidade nas políticas de Património Cultural Imaterial contemporâneas. Tendo como referência a minha pesquisa sobre festas do Espírito Santo (ou festas do Divino) nos Açores, na América do Norte e no Brasil, em particular – no caso do Brasil – em São Luís (Maranhão) defenderei três ideias e deixarei uma interrogação. A primeira ideia é que a festa não pode ser entendida sem ideias sobre comunidade, mesmo que coloquemos algumas aspas e especificações em “comunidade”. A segunda é que essas ideias, embora importantes, são insuficientes para dar conta das dinâmicas investidas na festa. De facto, para além de ideias sobre comunidade, a festa mobiliza e depende de um conjunto de outras agencialidades – de pessoas, redes de pessoas e especialistas rituais – que não podem ser subsumidas por ideias de comunidade. Partindo desta segunda ideia, proporei uma terceira ideia, assente numa reavaliação mais realista da importância que na festa têm ideias sobre comunidade. Quanto à interrogação é esta: será que abordar a festa por referência exclusiva aos agentes humanos – pessoas, redes de pessoas, comunidades, etc. – não deixa de lado aspectos importantes da festa? Sendo menos relevante para muitas festas “secularizadas”, esta interrogação é vital para festas com uma motivação e um conteúdo religiosos.

Cultural journalism in a digital environment : new models, practices and possibilities

Both culture coverage and digital journalism are contemporary phenomena that have undergone several transformations within a short period of time. Whenever the media enters a period of uncertainty such as the present one, there is an attempt to innovate in order to seek sustainability, skip the crisis or find a new public. This indicates that there are new trends to be understood and explored, i.e., how are media innovating in a digital environment? Not only does the professional debate about the future of journalism justify the need to explore the issue, but so do the academic approaches to cultural journalism. However, none of the studies so far have considered innovation as a motto or driver and tried to explain how the media are covering culture, achieving sustainability and engaging with the readers in a digital environment. This research examines how European media which specialize in culture or have an important cultural section are innovating in a digital environment. Specifically, we see how these innovation strategies are being taken in relation to the approach to culture and dominant cultural areas, editorial models, the use of digital tools for telling stories, overall brand positioning and extensions, engagement with the public and business models. We conducted a mixed methods study combining case studies of four media projects, which integrates qualitative web features and content analysis, with quantitative web content analysis. Two major general-interest journalistic brands which started as physical newspapers – The Guardian (London, UK) and Público (Lisbon, Portugal) – a magazine specialized in international affairs, culture and design – Monocle (London, UK) – and a native digital media project that was launched by a cultural organization – Notodo, by La Fábrica – were the four case studies chosen. Findings suggest, on one hand, that we are witnessing a paradigm shift in culture coverage in a digital environment, challenging traditional boundaries related to cultural themes and scope, angles, genres, content format and delivery, engagement and business models. Innovation in the four case studies lies especially along the product dimensions (format and content), brand positioning and process (business model and ways to engage with users). On the other hand, there are still perennial values that are crucial to innovation and sustainability, such as commitment to journalism, consistency (to the reader, to brand extensions and to the advertiser), intelligent differentiation and the capability of knowing what innovation means and how it can be applied, since this thesis also confirms that one formula doesn´t suit all. Changing minds, exceeding cultural inertia and optimizing the memory of the websites, looking at them as living, organic bodies, which continuously interact with the readers in many different ways, and not as a closed collection of articles, are still the main challenges for some media.

An essay on agile project management practices

The main goals for the current dissertation is to research on how practices and concepts from Agile Project Management can be applied in a non-IT context and to discover which aspects should be considered when deciding if whether an Agile approach should be implemented or not. Previous studies reflect on the adoption for the identified context. However, the recognition of these practices and concepts by the Project Management field of studies still remains unresolved. The adoption of Agile Project Management emerges as a manifestation against traditional approaches, mainly due to their inability of accepting requirements’ changes. Therefore, these practices and concepts can be considered in order to reduce the risks concerning the increase of competition and innovation – which does not apply to the IT sector solely. The current study reviews the literature on Agile Project Management and its adoption across different sectors in order to assess which practices and concepts can be applied on a non-IT context. Nine different methods are reviewed, where two of these show a higher relevance – Scrum and Extreme Programming. The identified practices and concepts can be separated into four different groups: Cultural and Organizational Structures, Process, Practices, and Artefacts. A framework based on the work by Boehm & Turner in 2004 is developed in order to support the decision of adopting agile methods. A survey intended for project managers was carried in order to assess the implementation of the identified practices and concepts and to evaluate which variables have the highest importance on the developed decision support framework. It is concluded that New Product Development is the project type with the highest potential to implement an agile approach and that the Project Final Product’s Innovativeness, Competitiveness, and the Project Member’s Experience and Autonomy are the most important aspects to consider an implementation of an Agile approach.

A indústria de defesa na cooperação Brasil-Portugal

The global dynamics of alliances are strongly determined by the level of cooperation among states. This cooperation can be embodied in various aspects, but the level of defense and security cooperation becomes usually more doctrinal and lasting. By the nature of sovereignty that instills in the bilateral relationship, cooperation at defense and security level can leverages other forms of cooperation. The circumstances and relational balance between Brazil and Portugal seem to evolve towards distancing opportunities, despite they are culturally and institutionally untainted. The economic dynamics, the strategic projection in global sustainability terms, the scale and ambition of Brazilian regional leadership, contrasts with the actual context of Portugal, distancing himself both on the stage where they operate. On the other hand, the historical and cultural roots, the language, the affinity of the peoples of CPLP and some opportunities for economic niches, trend to attract both countries. The condition of Portugal in NATO and Europe, coupled with the ability to export technical and human resources to value-added for Brazil, seems also to become approaching factors. On the balance of these dynamics, there is a set of exogenous factors (economic, external global relations matrix, regional stability, among others), which are not always controlled by any of both countries. These factors call for strong capacity for foresight analysis and decision making, with the inherent risk. There is cooperation vectors that are not apparently penalized by geographic distance, or by the difference of realities. Among these vectors we shall highlight synergies in technological niches, highly tradable goods and, mostly, using the domain of dual technologies. The thirteen niches herein identified are: Monitoring, Navigation, Command and Control, Electronics, Optoelectronics, Communication and remote sensing, Information Technologies, Flight Simulation, Specialized Training, Fiber Optic Sensors, Materials Engineering, Nanotechnology and Communications. Cumulating with identified opportunities in traditional relational framework, both countries are growing (in geography and economic terms) into the Atlantic, making it a central element in the bilateral approach. By being at the same time a growing stage of disputes and which stability tends to be threatened, it will be done an analysis of these synergistic vectors, superimposed on the impact on Atlantic securitization process.

Climatic aspects of the Amazonian Tropical Forest

The purpose of this study is to analyse the climatic aspects of the data collected in a forest site in comparison with conventional data obtained at different sites, such as clearing, rural an urban areas. The results showed that diverse climatic conditions do exist among the sites: the urban site showed higher temperature and lower relative humidity. In addition, evapotranspiration (potential and actual rates) was computed from the forest data set, using the classical Penman-Monteith's equation. The actual evapotranspiration is 30% of the potential value during dry period and seems to be almost constant during the whole year (tipically 2.0 to 2.5 mm day-1).

The effects of cultural heritage on residential property values: Evidence from Lisbon, Portugal

This paper examines the impact of historic amenities on residential housing prices in the city of Lisbon, Portugal. Our study is directed towards identifying the spatial variation of amenity values for churches, palaces, lithic (stone) architecture and other historic amenities via the housing market, making use of both global and local spatial hedonic models. Our empirical evidence reveals that different types of historic and landmark amenities provide different housing premiums. While having a local non-landmark church within 100 meters increases housing prices by approximately 4.2%, higher concentrations of non-landmark churches within 1000 meters yield negative effects in the order of 0.1% of prices with landmark churches having a greater negative impact around 3.4%. In contrast, higher concentration of both landmark and non-landmark lithic structures positively influence housing prices in the order of 2.9% and 0.7% respectively. Global estimates indicate a negative effect of protected zones, however this significance is lost when accounting for heterogeneity within these areas. We see that the designation of historic zones may counteract negative effects on property values of nearby neglected buildings in historic neighborhoods by setting additional regulations ensuring that dilapidated buildings do not damage the city’s beauty or erode its historic heritage. Further, our results from a geographically weighted regression specification indicate the presence of spatial non-stationarity in the effects of different historic amenities across the city of Lisbon with variation between historic and more modern areas.

As Necrópoles alto-medievais do concelho de Cascais : séculos VI e VII

A presente dissertação teve como finalidade estudar os testemunhos arqueológicos respeitantes às práticas funerárias levadas a cabo no atual concelho de Cascais durante os séculos VI e VII. As necrópoles em estudo são sítios bem conhecidos pelos investigadores, uma vez que a descoberta de algumas é precoce, datando de finais do século XIX. Com este trabalho, pretendeu-se introduzir uma série de componentes que a investigação privilegia atualmente, sobretudo no que respeita às vivências nos espaços rurais entre o fim do Império Romano e o domínio muçulmano na Península Ibérica. A investigação desenvolvida baseou-se no estudo preliminar das coleções osteológicas de quatro das cinco necrópoles, bem como na prospeção e no levantamento arqueológicos. O inventário antropológico teve como objetivo apurar o número mínimo de indivíduos por necrópole e por sepultura e fazer uma caracterização básica do sexo e da idade dos inumados. A prospeção assentou na análise das fontes bibliográficas sobre os sítios. Os trabalhos de campo desenvolveram-se no sentido de apurar o estado de conservação dos vestígios, na eventual identificação de outros novos e no consequente levantamento gráfico e fotográfico dos mesmos. Foi igualmente examinado o espólio cerâmico e metálico recolhido aquando da escavação das necrópoles, de modo obter uma visão abrangente sobre os rituais funerários e a estabelecer cronologias mais precisas. Embora se trate de um estudo limitado devido à inexistência de um registo mais rigoroso dos vestígios, foi possível tirar algumas conclusões e constatar alguns padrões. Os resultados possibilitaram apontar um conjunto de condições que se repetem nos locais onde estes cemitérios se implantam, percebendo dinâmicas em relação a fatores de caráter natural e de carácter antrópico. Também se apurou que existiriam diferentes formas de organizar os espaços funerários e que estes seriam constituídos por sepulturas muito diversas do ponto de vista construtivo. Além disso, começa-se a desvelar as razões para a reutilização de sepulturas para vários enterramentos e a entender a forma como as comunidades rurais conduziam os rituais funerários, ainda muito enraizados numa antiga matriz pagã.

Contributos para um Plano de Preservação no Arquivo Intermédio da Câmara Municipal de Sintra

Os aquivos municipais preservam a documentação definida legalmente como de conservação permanente, a memória e a experiência da Administração Local e, como tal, são agentes imprescindíveis à preservação dos direitos municipais. A preocupação com a preservação da documentação deve constituir um dos pilares principais do arquivo desde o início da sua criação. E não somente quando a documentação é transferida para o Arquivo Histórico, deve estar presente durante todo o ciclo de vida da documentação. Com a criação do PARAM (Programa de Apoio à Rede de Arquivos Municipais), os arquivos municipais ganham um novo incremento, não só a nível técnico como a nível financeiro, sendo-lhe reconhecido o seu devido valor e importância, na sua dupla dimensão, administrativa e cultural. Uma das funções do arquivo é disponibilizar a documentação que têm à sua guarda e que se encontra armazenada nos depósitos. Para tal é fundamental criar condições a curto, a médio e a longo prazo, implementando toda uma política integrada de preservação, envolvendo os demais serviços da instituição, fazendo-se o controlo do estado de conservação da documentação, aplicando uma metodologia da manipulação de documentos para disponibilizá-los tanto aos clientes internos como externos. O Arquivo Intermédio da Câmara Municipal de Sintra não possui plano de preservação documental, nem ações de preservação extensíveis aos objetos digitais, daí que a presente tese de mestrado pretenda ser um proposta de um contributo de um plano de preservação, que tenha em consideração os demais aspetos desde a localização e construção ou manutenção do edifício onde se encontra o arquivo, questões de segurança, de controlo ambiental, de luz/fotodegradação, o controlo integrado de pragas, danos causados por água, os desastres naturais, a poluição, a limpeza e vigilância na zona de depósitos e armazenamento e manuseamento dos documentos.

Does the existing Corporate Governance structure and strict Legal Framework, affect the strategic decisionmaking of CEOs?

This thesis is a case study on Corporate Governance and Business Ethics, using the Portuguese Corporate Law as a general setting. The thesis was conducted in Portugal with illustrations on past cases under the Business Judgment Rule of the State of Delaware, U.SA along with illustrations on current cases in Portugal under the Portuguese Judicial setting, along with a comparative analysis between both. A debate is being considered among scholars and executives; a debate on best practices within corporate governance and corporate law, associated with recent discoveries of unlawful investments that lead to the bankruptcy of leading institutions and an aggravation of the crisis in Portugal. The study aimed at learning possible reasons and causes for the current situation of the country’s corporations along with attempts to discover the best way to move forward. From the interviews and analysis conducted, this paper concluded that the corporate governance structure and legal frameworks in Portugal were not the sole influencers behind the actions and decisions of Corporate Executives, nor were they the main triggers for the recent corporate mishaps. But it is rather a combination of different factors that played a significant role, such as cultural and ethical aspects, individual personalities, and others all of which created gray areas beyond the legal structure, which in turn accelerated and aggravated the corporate governance crisis in the country.

Acordo Ortográfico da Língua Portuguesa e o significado da lusofonia: análise comparativa

Tese de Doutoramento em Ciências da Comunicação (área de especialização em Sociologia da Comunicação e da Informação).