Clinical and laboratorial study of 19 cases of mucopolysaccharidoses

The mucopolysaccharidoses (MPS) are a heterogeneous group of inborn errors of lysosomal glycosaminoglycan (GAG) metabolism. The importance of this group of disorders among the inborn errors of metabolism led us to report 19 cases. METHOD: We performed clinical, radiological, and biochemical evaluations of the suspected patients, which allowed us to establish a definite diagnosis in 19 cases. RESULTS: Not all patients showed increased GAG levels in urine; enzyme assays should be performed in all cases with strong clinical suspicion. The diagnosis was made on average at the age of 48 months, and the 19 MPS cases, after a full clinical, radiological, and biochemical study, were classified as follows: Hurler -- MPS I (1 case); Hunter -- MPS II (2 cases); Sanfilippo -- MPS III (2 cases); Morquio -- MPS IV (4 cases); Maroteaux-Lamy -- MPS VI (9 cases); and Sly -- MPS VII (1 case). DISCUSSION: The high relative frequency of Maroteaux-Lamy disease contrasts with most reports in the literature and could express a population variability.

Os romances juvenis de Álvaro Magalhães

With his works translated in countries such as Spain, France and Brazil, Álvaro Magalhães is not only one of the great writers of Portuguese and Lusophone juvenile fiction, but also one of the most prominent names in European and world literature. Through the various thematic and narrative resources that he employs in the creation of a unique language, among the multiple and unusual senses of serious and humorous, among the elements of various domains of knowledge and universal culture that he invokes and contrasts, this author has a unique place in universe of novels aimed primarily at young readers.

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.

Geomorphology and land development in the Maraca area of northern Roraima, Brazil.

The geomorphological materials and forms of the Maraca area of Roraima, Brazil are described, an their sgnificance for land development examined. Significant contrasts are noted in areas presently under rainforest and savanna vegetation. Lateritic gravels and extensive shetwash accumulations in savanna areas constrast with incipient or absent plinthite development, few gravels and limited evidence of colluvium under rainforest. Terrain is in general relatively highly-dissected. Slope profiles are characterised, particularly within the savanna zone, by a relatively steep lower concavity. These contrasts are sharply-demarcated by the present savanna/rainforest bondary, unexpectedly in view of the generally accepted hypothesis of repeated contraction an expansion of Amazonian rainforest throughout the Pleistocene. It is concluded that geomorphological conditions in the Maraca area are not favorable for land develoment.

Changes in population and land use over time in the Ecuadorian Amazon

This paper draws upon a detailed longitudinal survey of households living on agricultural plots in the northern three provinces of the Ecuadorian Amazon, the principal region of colonization by migrants in Ecuador since the 1970s. Following the discovery of petroleum in 1967 near what has subsequently come to be the provincial capital and largest Amazonian city of Lago Agrio, oil companies built roads to lay pipelines to extract and pump oil across the Andes for export. As a result, for the past 30 years over half of both Ecuador's export earnings and government revenues have come from petroleum extracted from this region. But the roads also facilitated massive spontaneous in-migration of families from origin areas in the Ecuadorian Sierra, characterized by minifundia and rural poverty. This paper is about those migrants and their effects on the Amazonian landscape. We discuss the data collection methodology and summarize key results on settler characteristics and changes in population, land use, land ownership, technology, labor allocation, and living conditions, as well as the relationships between changes in population and changes in land use over time. The population in the study region has been growing rapidly due to both natural population growth (high fertility) and in-migration. This has led to a dramatic process of subdivision and fragmentation of plots in the 1990's, which contrasts with the consolidation of plots that has occurred in most of the mature frontier areas of the Brazilian Amazon. This fragmentation has led to important changes in land tenure and land use, deforestation, cattle raising, labor allocation, and settler welfare.

Momentos de inovação: emergência e evolução das protonarrativas em terapia narrativa

Dissertação de mestrado em Psicologia Aplicada

Modelos de negocio orientados al consumidor en los cibermedios españoles

La viabilidad de los cibermedios y los modelos de negocio que la sustenten son ya un tema  clásico  de  la  literatura  académica  y  profesional  en  el  ámbito  del  periodismo  electrónico. Consideramos relevante examinar las estrategias de ingresos orientadas al consumidor  de  los  cibermedios  españoles  en  un  contexto  de  madurez  de  la  prensa  digital que contrasta con la aguda crisis económica que vive en estos momentos el país.  Las  conclusiones  de  nuestro  estudio  señalan  la  convivencia  de  dos  modelos  de  negocio diametralmente distintos entre los cibermedios más vistos y de referencia en Internet en España y el establecimiento de estrategias de ‘suma de valor’ en aquellos cibermedios que han establecido sistemas de pago por consumo de contenidos.

O filme “A Gaiola Dourada”: reflexões sobre o regresso em força da emigração portuguesa e a ‘portugalidade’ de uma gaiola (cada vez menos) dourada

A história de um casal de emigrantes portugueses em França bateu, no verão de 2013, recordes de audiência nas salas de cinema. O filme “A Gaiola Dourada”, de Ruben Alves, recuperou a temática da emigração portuguesa, numa altura em que esta atingiu o boom registado nos anos 1960. Recorrendo ao seu percurso, o realizador refere-se à ‘portugalidade’ como alegada ‘pertença a Portugal’, que assume como um cliché, e utiliza de forma humorística vários estereótipos associados aos emigrantes portugueses, através dos quais é mostrada alguma vergonha que os filhos dos emigrantes sentem em relação ao comportamento dos pais, trazendo ao de cima os contrastes com a sociedade onde vivem. A grande ficção reside no regresso ao país de origem, concretizando o sonho da grande maioria dos emigrantes, mas subvertendo a lógica: no filme, não são os pais que voltam a Portugal, mas os filhos, que aparentemente pouco se identificam com o país dos progenitores. “A Gaiola Dourada” reintroduziu o debate sobre a emigração em Portugal, aproveitando a crise económica para refletir sobre o seu regresso em força, bem como traçar o perfil dos novos emigrantes. Será que os portugueses na ‘diáspora’ ainda reavivam a chama ‘lusitana’ (Gonçalves, 2009)? E será que a partilha do nome ‘Portugal’ basta para sublinhar uma alegada identidade nacional (Sobral, 2012)?

Sistemas de controlo de gestão, empreendedorismo corporativo, estratégia e performance : contraste entre empresas familiares e não familiares

Mestrado em Contabilidade, Fiscalidade e Finanças Empresariais

Experiments in square lattice with a common treatment in all blocks

This paper deals with a generalization of square lattice designs, with k² treatments in blocks of k + 1 plots, the extra plot in each block receiving a standard treatment, the same for all blocks. The new design leads to lower variances for contrasts between adjusted treatment means

Effects of selection and drift on G matrix evolution in a heterogeneous environment: a multivariate Qst-Fst Test with the freshwater snail Galba truncatula.

Unraveling the effect of selection vs. drift on the evolution of quantitative traits is commonly achieved by one of two methods. Either one contrasts population differentiation estimates for genetic markers and quantitative traits (the Q(st)-F(st) contrast) or multivariate methods are used to study the covariance between sets of traits. In particular, many studies have focused on the genetic variance-covariance matrix (the G matrix). However, both drift and selection can cause changes in G. To understand their joint effects, we recently combined the two methods into a single test (accompanying article by Martin et al.), which we apply here to a network of 16 natural populations of the freshwater snail Galba truncatula. Using this new neutrality test, extended to hierarchical population structures, we studied the multivariate equivalent of the Q(st)-F(st) contrast for several life-history traits of G. truncatula. We found strong evidence of selection acting on multivariate phenotypes. Selection was homogeneous among populations within each habitat and heterogeneous between habitats. We found that the G matrices were relatively stable within each habitat, with proportionality between the among-populations (D) and the within-populations (G) covariance matrices. The effect of habitat heterogeneity is to break this proportionality because of selection for habitat-dependent optima. Individual-based simulations mimicking our empirical system confirmed that these patterns are expected under the selective regime inferred. We show that homogenizing selection can mimic some effect of drift on the G matrix (G and D almost proportional), but that incorporating information from molecular markers (multivariate Q(st)-F(st)) allows disentangling the two effects.

Analog Modelling of N-S anticlines of the External Sierras (Jaca basin, Southern Pyrenees)

Report for the scientific sojourn carried out at the Uppsala Universitet, Sweden, from April to July the 2007. Two series of analogue models are used to explore ductile-frictional contrasts of the basal décollement in the development of oblique and transverse structures simultaneously to thin-skinned shortening. These models simulate the evolution of the Central External Sierras (Southern Pyrenees, Spain), which constitute the frontal emerging part of the southernmost Pyrenean thrust sheet. They are characterized by the presence of transverse N-S to NW-SE anticlines, which are perpendicular to the Pyrenean structural trend and developed in the hangingwall of the Santo Domingo thrust system, detaching on an unevenly distributed Triassic materials (evaporitic-dolomitic interfingerings). Model setup performs a décollement made by three patches of silicone neighbouring pure brittle sand. Model series A test the thickness ratio between overburden and décollement. Model series B test the width of frictional detachment areas. Model results show how deformation reaches further in areas detached on ductile layer whereas frictional décollement areas assimilate the strain by means of an additional uplift. This replicates the structural style of Central External Sierras: higher structural relief of N-S anticlines with regard to orogen-parallel structures, absence of a representative ductile décollement in the core, tilting towards the orogen and foreland-side closure not thrusted by the frontal emerging South-Pyrenean thrust.

Endogenous Price Flexibility and Optimal Monetary Policy

Much of the literature on optimal monetary policy uses models in which the degree of nominal price flexibility is exogenous. There are, however, good reasons to suppose that the degree of price flexibility adjusts endogenously to changes in monetary conditions. This paper extends the standard New Keynesian model to incorporate an endogenous degree of price flexibility. The model shows that endogenising the degree of price flexibility tends to shift optimal monetary policy towards complete inflation stabilisation, even when shocks take the form of cost-push disturbances. This contrasts with the standard result obtained in models with exogenous price flexibility, which show that optimal monetary policy should allow some degree of inflation volatility in order to stabilise the welfarerelevant output gap.

Local Currency Pricing, Foreign Monetary Shocks and Exchange Rate Policy

The implications of local currency pricing (LCP) for monetary regime choice are analysed for a country facing foreign monetary shocks. In this analysis expenditure switching is potentially welfare reducing. This contrasts with the existing LCP literature, which focuses on productivity shocks and thus analyses a world where expenditure switching is welfare enhancing. This paper shows that, when home and foreign producers follow LCP, expenditure switching is absent and a floating rate is preferred by the home country. But when only home producers follow LCP, expenditure switching is present and a fixed rate can be welfare enhancing for the home country.

Institutions and prosperity

This article reviews ‘Pillars of Prosperity’ by Timothy Besley and Torsten Persson and ‘Why Nations Fail’ by Daron Acemoglu and James Robinson. Both books are focussed on the role of institutions in determining the wealth of nations and the review compares and contrasts the different approaches contained in the two texts. The review also attempts to locate the texts within the broader literature in development and political economics and to link them to other recent work in these areas.