Estudo clínico e bioquímico de 28 pacientes com mucopolissacaridose tipo VI

Realizamos um estudo observacional de pacientes com mucopolissacaridose tipo VI, com o objetivo de determinar o perfil epidemiológico, clínico e bioquímico de um grupo de pacientes sul-americanos a fim de contribuir em estudos futuros de correlação genótipo-fenótipo e de avaliação de protocolos clínicos. Os critérios de inclusão foram: ter 4 anos ou mais e confirmação bioquímica da doença (níveis reduzidos da atividade da ARSB, aumento de GAGs urinários e atividade normal de outra sulfatase). Os critérios de exclusão foram: terapia com reposição enzimática atual ou prévia ou ter realizado transplante de medula óssea. Foram avaliados 28 pacientes por anamnese, exame físico, acocardiograma, eletrocardiograma, avaliação oftalmológica, medidas de glicosaminoglicanos urinários e da atividade da N-acetilgalactosamina-4-sulfatase em leucócitos. A amostra estudada tinha 92,9% de brasileiros, sendo 53,8% da região sudeste. No momento da avaliação, a média de idade foi de 97,1 meses e a média de idade ao diagnóstico foram de 48,4 meses. Em 88% da amostra os sintomas iniciaram com menos de 36 meses e em 27% das famílias houve relato de consangüinidade entre os pais. A média de peso e estatura ao nascimento foi de 3481 gramas e 51,3 centímetros, respectivamente. Da amostra, 57,1% nasceram de parto vaginal. Todos apresentavam alguma alteração ecocardiográfica, bem como opacificação corneana. As manifestações clínicas mais freqüentes foram: baixa estatura, opacificação corneana, facies grosseira, contraturas articulares e mãos em garra. A média da atividade enzimática em leucócitos foi de 5,4 nmoles/h x mg proteína e a excreção urinária de glicosaminoglicanos foi, em média, 7,9 vezes superior ao normal. O número de manifestações clínicas citadas não apresentou correlação significativa com a idade, com a excreção urinária de GAGs ou com a atividade enzimática em leucócitos. Também não houve correlação significativa entre a excreção urinária de GAGS e a atividade enzimática. Concluímos que a MPS VI é uma patologia com alta morbidade e que, comparados com a literatura, os pacientes da nossa amostra têm um diagnóstico tardio e maior freqüência de alterações cardiológicas.

A complexidade da gestão em processos de internacionalização: como a cultura Nacional impacta o processo decisório de gestores brasileiros expatriados - o caso de uma empresa estatal do setor de energia

In a world where the companies are trying to compete on a global scale, the positioning strategies direct them to go beyond the geographical boundaries of their base countries, seeking for other markets, as a way to keep globally competitive. These conditions make crucial the roles performed by their managers, exposed to different contexts, mainly economical, political, social and cultural and to face the task to manage enterprises like a challenge, probably bigger than the one found on their base countries. This speech tries to examine how much expatriated Brazilian managers feel the influence of local cultural factors in their decisional processes, taking into consideration that they are embedded in their cultural home countries, and they can suffer an impact by the culture of other countries. With this research it is intended to gather empirical material that will supply clues and allow to advance on studies that will make a relationship between decisional process and local culture, through a case study in a Brazilian state-owned energy company and its subsidiaries in South American countries, hoping that the mentioned studies will somehow contribute to a better understanding of the problems that have been stressed in the last decades. Also considering that the vast majority of the theoretical and empirical material about this matter has its own source on the western developed countries and are, in this way, culture-bounded, we hope to increase the production of studies and researches applied to the Latin American reality, mainly the Brazilian one.

Os recursos físicos para a educação formal na América Latina: uma análise de sua distribuição

Este estudo examina o modo como se encontram distribuídos os recursos humanos e materiais, destinados ao ensino formal de 1o e 2o Graus, em amostras de escolas de cinco países da América do Sul (Argentina, Bolívia, Brasil, Paraguai e Peru). Mediante o emprego de uma metodologia quantitativa, a distribuição dos recursos é estudada segundo a localizaçao geográfica, a dependência administrativa, o nível de ensino e a origem social da clientela das escolas. A ·análise dos dados indica a existência de flagrantes disparidades na dotação de recursos as escolas, cujas causas devem ser buscadas no sistema social mais amplo, e não apenas no contexto interno do sistema de ensino de cada país.

Fundamentos de cartografia

Apresenta fundamentos de cartografia e sua aplicação no Geoprocessamento. Define os conceitos e aplicações de geóide, elipsóide, datum, sistema de referência, South American Datum (SAD-69), Sistema de Referência Geocêntrico para as Américas (SIRGAS 2000), elipsóide GRS80, elipsóide WGS84. Exemplos de utilização de sistemas de referência distintos e problemas relacionados.

Antígenos da forma amastigota de Leishmania chagasi identificados por dupla varredura da biblioteca de cDNA

Control of human visceral leishmaniasis in endemic regions is hampered in part by the lack of knowledge with respect of the role reservoirs and vector. In addition, there is not yet an understanding of how non-symptomatic subclinical infection might influence the maintenance of infection in a particular locality. Of worrisome is the limited accessibility to medical care in places with emerging drug resistance. There is still no available protective vaccine either for humans or other reservoirs. Leishmania species are protozoa that express multiple antigens which are recognized by the vertebrate immune system. Since there is not one immunodominant epitope recognized by most hosts, strategies must be developed to optimize selection of antigens for prevention and immunodiagnosis. For this reason, we generated a cDNA library from the intracellular amastigote form of Leishmania chagasi, the causative agent of South American visceral leishmaniasis. We employed a two-step expression screen of the library to systematically identify T and T-dependent B cell antigens. The first step was aimed at identifying the largest possible number of clones producing an epitope-containing polypeptide with a pool of sera from Brazilians with documented visceral leishmaniasis. After removal of clones encoding heat shock proteins, positive clones underwent a second step screen for their ability to cause proliferation and IFN-γ responses of T cells from immune mice. Six unique clones were selected from the second screen for further analysis. The clones encoded part of the coding sequence of glutamine synthetase, transitional endoplasmic reticulum ATPase, elongation factor 1γ, kinesin K-39, repetitive protein A2, and a hypothetical conserved protein. Humans naturally infected with L. chagasi mounted both cellular and antibody responses to these protein Preparations containing multiple antigens may be optimal for immunodiagnosis and protective vaccines against Leishmania

Pleistocene mammals from the southern Brazilian continental shelf

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

Crystallization, preliminary X-ray analysis and molecular-replacement solution of haemoglobin-II from the fish matrinxa (Brycon cephalus)

Haemoglobins constitute a set of proteins with interesting structural and functional properties, especially when the two large animal groups reptiles and fishes are focused on. Here, the crystallization and preliminary X-ray analysis of haemoglobin-II from the South American fish matrinxa (Brycon cephalus) is reported. X-ray diffraction data have been collected to 3.0 Angstrom resolution using synchrotron radiation (LNLS). Crystals were determined to belong to space group P2(1) and preliminary structural analysis revealed the presence of two tetramers in the asymmetric unit. The structure was determined using the standard molecular-replacement technique.

Injúrias causadas por mosca-das-frutas sul-americana em cultivares de videira

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

Cryptic lineages and Pleistocene population expansion in a Brazilian Cerrado frog

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

The role of nitric oxide in the regulation of the systemic and pulmonary vasculature of the rattlesnake, Crotalus durissus terrificus

The functional role of nitric oxide (NO) was investigated in the systemic and pulmonary circulations of the South American rattlesnake, Crotalus durissus terrificus. Bolus, intra-arterial injections of the NO donor, sodium nitroprusside (SNP) caused a significant systemic vasodilatation resulting in a reduction in systemic resistance (Rsys). This response was accompanied by a significant decrease in systemic pressure and a rise in systemic blood flow. Pulmonary resistance (Rpul) remained constant while pulmonary pressure (Ppul) and pulmonary blood flow (Qpul) decreased. Injection of L-Arginine (L-Arg) produced a similar response to SNP in the systemic circulation, inducing an immediate systemic vasodilatation, while Rpul was unaffected. Blockade of NO synthesis via the nitric oxide synthase inhibitor, L-NAME, did not affect haemodynamic variables in the systemic circulation, indicating a small contribution of NO to the basal regulation of systemic vascular resistance. Similarly, Rpul and Qpul remained unchanged, although there was a significant rise in Ppul. Via injection of SNP, this study clearly demonstrates that NO causes a systemic vasodilatation in the rattlesnake, indicating that NO may contribute in the regulation of systemic vascular resistance. In contrast, the pulmonary vasculature seems far less responsive to NO.

Development and characterization of microsatellite loci in the marsh deer (Blastocerus dichotomus Cervidae)

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

Da Amazônia ao Pacífico cruzando os Andes

A Área Amazônica (AA) é compartilhada por oito países sul-americanos independentes. Desde os tempos coloniais sentiu-se a necessidade da construção de uma via de união da AA com o Oceano Pacífico. Mas os Andes, em termos técnicos e econômicos, foram barreira insuperável até as últimas décadas do século XIX. Nos últimos anos, para o Brasil - país que tem perto de 60% da AA -, esta via vem se tornando cada vez mais importante em termos de exportação, especialmente para o mercado japonês, de grãos, madeira, polpa de madeira etc., ao mesmo tempo em que o Japão também deseja acesso direto à AA. Brasil e Peru, segundo país da AA, com a maior e mais ocidental costa no Pacífico, têm desenvolvido projetos para a construção de uma estrada, contando para isso com apoio financeiro oferecido pelo Japão em diversas oportunidades. Tais projetos tentam conciliar não apenas os interesses de Brasil e Peru, mas também os da Bolívia, país mediterrâneo que poderia finalmente ter acesso livre à costa do Pacífico. Os Estados Unidos, porém, opõem-se a construção da mencionada via e, o projeto está a espera de que o financiamento anteriormente aventado permita sua concretização. Parece que, na construção da projetada estrada, interesses estratégicos das grandes potências mundiais estão envolvidos.

Fission track and U-Pb in situ dating applied to detrital zircon from the Vale do Rio do Peixe Formation, Bauru Group, Brazil

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

O mundo criado pelas imagens: paisagens e espaços coloniais na obra do holandês Frans Post

The aims of this dissertation is to study formation of the Dutch view seeing the colonial scenery in screens by Frans Post, as well as, to perceive a colonial world constitution through landscape paintings by him with his natural and human representation. The artist was the first to portray South American views, after he landed in Pernambuco with retinue of Dutch governor of colony, John Maurice, Prince of Nassau-Siegen. Post, by his 24 years old, was designated to represent for Dutch people their colony. The text reflects on visual construction of natural and human aspects in landscapes by Dutchman and how that aspects were included in colonizer imaginary about the strange world of America. European (Dutch) look about their conquered possessions in the New World was charged with exoticism and imagination. In order to understand that view, it`s paramount to study imaginary pictures reared by Frans Post, on his return to the Netherlands, and notions of landscape and exotic, wild and unspoiled nature which the Dutch people had when they thought about the Dutch colony in America. Our principal (visual) sources of research are six paintings: Vista da Sé de Olinda (1662), Vista das ruínas de Olinda (undated), Engenho (undated), Engenho (1660), Vista da cidade Maurícia e do Recife (1653), e Paisagem com rio e tamanduá (1649), all these canvases were painted when Frans Post returned to Europe. We seek to work through a methodology that focuses on investigation of primary visual and textual material, because these textual and pictorial representations reflect the 17th-century colonial view of colonial history themes of the - here called - Dutch America

Estruturas tectônicas cenozóicas na porção leste da Bacia Potiguar - RN

This thesis encompasses the integration of geological, geophysical, and seismological data in the east part of the Potiguar basin, northeastern Brazil. The northeastern region is located in South American passive margin, which exhibits important areas that present neotectonic activity. The definition of the chronology of events, geometry of structures generated by these events, and definition of which structures have been reactivated is a necessary task in the region. The aims of this thesis are the following: (1) to identify the geometry and kinematics of neotectonic faults in the east part of the Potiguar basin; (2) to date the tectonic events related to these structures and related them to paleoseismicity in the region; (3) to present evolutional models that could explain evolution of Neogene structures; (4) and to investigate the origin of the reactivation process, mainly the type of related structure associated with faulting. The main type of data used comprised structural field data, well and resistivity data, remote sensing imagery, chronology of sediments, morphotectonic analysis, x-ray analysis, seismological and aeromagnetic data. Paleostress analysis indicates that at least two tectonic stress fields occurred in the study area: NSoriented compression and EW-oriented extension from the late Campanian to the early Miocene and EW-oriented compression and NS-oriented extension from the early Miocene to the Holocene. These stress fields reactivated NE-SW- and NW-SE-trending faults. Both set of faults exhibit right-lateral strike-slip kinematics, associated with a minor normal component. It was possible to determine the en echelon geometry of the Samambaia fault, which is ~63 km long, 13 km deep, presents NE-SW trend and strong dip to NW. Sedimentfilled faults in granite rocks yielded Optically Stimulated Luminescence (OSL) and Single-Aliquot Regeneration (SAR) ages at 8.000 - 9.000, 11.000 - 15.000, 16.000 - 24.000, 37.000 - 45.500, 53.609 - 67.959 e 83.000 - 84.000 yr BP. The analysis of the ductile fabric in the João Câmara area indicate that the regional foliation is NE-SW-oriented (032o - 042o), which coincides with the orientation of the epicenters and Si-rich veins. The collective evidence points to reactivation of preexisting structures. Paleoseismological data suggest paleoseismic activity much higher than the one indicated by the short historical and instrumental record