Single crystals of GaSe were grown from the melt using 3-zone vertical Bridgman-Stockbarger system. In order to determine the doping effect, nitrogen and silicon ions were implanted to the grown crystals. Surface morphology and stoichiometry were examined using scanning electron microscope equipped with EDAX and structure properties were examined by x-ray diffraction technique. It was observed that the resulting ingot was stoichiometric and the structure was hexagonal. To identify the effects of ion implantation on the physical properties of the samples depending on annealing / electrical conductivity, hall measurements, current-voltage characteristics, photoconductivity and photoluminescence measurements were carried out in the temperature range of 100-450 K. Also spectral transmission measurements were carried out for all the samples at room temperature. It was observed that both N- and Si- implantation followed by annealing process decreased the resisitivity values from 107 to 103 (&amp / #61527 / -cm). Temperature dependent conductivity measurements were analyzed to deduce the dominant transport mechanisms. The trap levels were also investigated by the space charge limited currents (SCLC) measurements. The temperature dependence of hole concentrations showed that as-grown, N- and Si-implanted samples behave as partially compensated p-type semiconductors. Using suitable statistical method, transport parameters such as acceptor level, donor and acceptor concentrations were extracted from the experimental data. Trapping centers and recombination mechanisms were determined from the temperature dependent photoconductivity measurements by investigating the relation between photocurrent and illumination intensity. N- and Si- implantation effects on GaSe were also examined by spectral photoconductivity and transmission measurements. And lastly, radiative recombination mechanisms in as-grown GaSe were investigated through photoluminescence (PL) measurements and the information related to the structural defects, the exciton levels and the structure of the forbidden gap were investigated.

In this work, structural, electrical and optical characterization of as-grown, Ge-implanted, and annealed GaSe single crystals grown by using 3-zone vertical Bridgman-Stockbarger system, have been studied by carrying out X-ray Diffraction (XRD), electrical conductivity, Hall effect, photoconductivity, and spectral transmission measurements. The temperature dependent electrical conductivity of these samples have been measured between 100 and 400 K. As a result, it was observed that upon implanting GaSe with germanium following annealing process, the resistivity is reduced from 2.1x109 to 6.5x105 &amp / #937 / -cm. Also it was found that Ge-implantation followed by annealing at 500 oC increases the conductivity in exponential fashion. From the temperature dependent conductivities, the activation energies have been found to be 4, 34 and 314 meV for as-grown, 36 and 472 meV for as-implanted, and 39 and 647 meV for implanted and annealed at 500 oC GaSe single crystals. Using XRD measurements it was observed that there is an increase in peak intensities at specific annealing temperatures (300 and 500 C) and a decrease in higher annealing temperatures (700 C). Temperature dependent carrier concentration and Hall mobility measurement were performed in the temperature range of 230 - 410 and 100 - 400 K for as-grown and Ge-implanted and annealed GaSe samples, respectively. All of the samples in this study were found to be p-type with the help Hall measurements. In addition, the density of donor and acceptor atoms are found for each sample and results are compared with each other. In addition, using photoconductivity measurement the relation between photocurrent and illumination intensity and the character of photoconduction were determined. As a result it was found that while at specific temperature intervals impurity scatterings are dominant, in other intervals phonon scatterings start to dominate. Finally, in order to determine annealing dependent change of band gap of unimplanted and Ge-implanted GaSe samples at room temperature, the transmission measurement have been carried out as a optical characterization part of our study. As a consequence of this measurement it was observed that there is almost no considerable change in optical band gap of samples with increasing annealing temperatures for as-grown GaSe samples and a slight shift of optical band gap toward to high energy for Ge-implanted samples with annealing process.

O presente trabalho apresenta o estudo da esteira de turbinas eólicas de eixo horizontal, utilizando um modelo de turbina eólica testada em túnel de vento. Trata-se de uma turbina eólica de duas pás com 10 m de diâmetro, conhecida como UAE Phase VI, testada pelo NREL no túnel de vento NASA Ames. Desta turbina foi obtida a geometria e dados experimentais, como o coeficiente de empuxo, além das características do túnel de vento. Utilizando este experimento como modelo, são realizadas simulações com equações médias de Reynolds e modelo de turbulência k-ω SST, com o uso de malha móvel e abordagens permanente e transiente, com o software de volumes finitos Star-CCM+®. São empregadas malhas poliédricas em um domínio de grande extensão na esteira, para apenas uma velocidade do vento. São apresentados três modelos de esteira, dois analíticos e outro considerado numérico, para fins de comparação com os resultados simulados. Estes modelos de esteira tem o objetivo de descrever o campo de velocidade a jusante da turbina. Os dados de entrada dos modelos são os mesmos, retirados da simulação. Além dos modelos para o déficit de velocidade, são apresentados equacionamentos para se obter a intensidade de turbulência na esteira, com bons resultados. No modelo analítico de esteira PARK, a constante de decaimento é baixa, pois o caso estudado possui escoamento sem grandes perturbações. Verifica-se que esta constante deve variar ao longo da esteira para se ajustar ao campo médio de velocidades simulado. Outro modelo analítico proposto por Werle foi estudado, não apresentando boa concordância com a simulação, pois apresenta um rápido incremento da velocidade na linha central na esteira distante. Este modelo se ajustou bem com o campo não perturbado a montante da turbina. O modelo de viscosidade turbulenta é um modelo de esteira com equacionamento original de segunda ordem para obter um campo de velocidade. Contudo, é apresentado e calculado com uma simplificação para primeira ordem, obtendo-se somente a velocidade na linha de centro. Este modelo tem o melhor ajuste com a simulação. A metodologia adotada para simulação numérica do escoamento apresenta boa concordância com os resultados experimentais de CT, já os dados anemométricos medidos na esteira próxima não foram reproduzidos com a mesma qualidade, contudo aproximam-se de forma qualitativa. A comparação com os modelos de esteira da literatura confirma que o modelo de viscosidade turbulenta é o que mais se aproxima dos resultados da simulação. / This study presents wakes of horizontal axis wind turbines, using as model a wind turbine tested in wind tunnel. It is a two bladed wind turbine with 10 m diameter, known as UAE Phase VI, tested by NREL in the NASA Ames wind tunnel. From this experiment was obtained the geometry and measured data, as the thrust coefficient, and the characteristics of the wind tunnel. Using this experiment as model, simulations are performed with Reynolds Averaged Navier-Stokes equations and k-ω SST turbulence model, using moving mesh and permanent and transient approaches, with help of the finite volume software Star-CCM+®. Polyhedral meshes are employed in a domain with large extent downwind, for just one wind speed. There are presented three wake models, two analytical and other considered numerical, for purposes of comparison with simulated results. These wake models aims to describe the velocity field downstream of the turbine. The input data of the models are the outputs of the simulations. Besides the models for the velocity deficit, are given equations to derive the turbulence intensity on the wake, with good results. In the analytical PARK wake model, the decay constant is low, as the study case has low perturbed flow. It is noted that this constant should vary along the wake to fit the average speeds field simulated. Another analytical model proposed by Werle is presented, not showing good results when comparing with the simulation, since it shows a rapid increase in the centerline velocity on the far wake. This model fits well with the undisturbed field upwind of the turbine. The turbulent viscosity is a wake model with the original second order equation to obtain the velocity field. However, it is presented and calculated in a simplified way to first order, obtaining only the centerline velocity. This model has the best fit with the simulation. The methodology adopted for numerical simulation of the flow shows good agreement with the experimental results of CT, since the wind data measured on the near wake are not reproduced with the same quality, yet close to a qualitative manner. The comparison with the wake models from the literature confirms that the eddy viscosity wake model is closest to the simulation results.

Introdução: As mucopolisaccaridoses tipo I (MPS I), tipo II (MPS II) e tipo VI (MPS VI) são doenças lisossômicas (DL) para as quais está disponível a terapia de reposição enzimática (TRE) com laronidase, idursulfase e galsufase, respectivamente. Objetivo Primário: Analisar a frequência anual de intervenções médicas (número de consultas, internações, cirurgias, exames solicitados, medicamentos prescritos, equipamentos de uso crônico e outras formas de terapia) em uma amostra de pacientes brasileiros com MPS I, II e VI e, desta forma, contribuir para o conhecimento dos aspectos farmacoeconômicos relacionados a essas doenças. Metodologia: Estudo exploratório, retrospectivo, de base hospitalar, baseado em revisão de prontuário, com amostragem por conveniência, e que foi realizado em duas etapas (etapas 1 e 2). Um instrumento específico para a coleta de dados de ambas as etapas foi construído pela equipe do estudo, que é multidisciplinar. Os desfechos de interesse foram as frequências anuais de intervenções médicas (consultas, exames, cirurgias, internações, medicamentos utilizados, outras formas de terapia). A etapa 1 consistiu em estudo pré-experimental, realizado no Serviço de Genética Médica do Hospital de Clínicas de Porto Alegre (SGM-HCPA), e que comparou as variáveis de interesse, para o mesmo grupo de pacientes, entre o período pré e pós-TRE. Os critérios de inclusão dessa etapa foram: ter diagnóstico confirmado de MPS I; estar em acompanhamento regular no SGM-HCPA desde o diagnóstico; estar em TRE por pelo menos um ano; e não ter participado de ensaio clínico envolvendo TRE ou ter realizado transplante de células-tronco hematopoiéticas. A etapa 2 foi transversal, multicêntrica (centros incluídos: SGMHCPA, Departamento de Genética Médica da Universidade Estadual de Campinas - UNICAMP, Pontifícia Universidade Católica de Campinas – PUC-Campinas, e Departamento de Pediatria da Universidade Estadual do Rio de Janeiro - UERJ), e comparou as variáveis de interesse entre grupos diferentes de pacientes (aqueles recebendo TRE e aqueles não recebendo TRE). Para essa etapa, foram considerados somente os dados relativos a 2010, sendo os seguintes os critérios de inclusão dos pacientes: ter diagnóstico confirmado de MPS I, II e VI; não estar participando de nenhum ensaio clínico envolvendo TRE ou ter realizado transplante de células-tronco hematopoiéticas; estar em TRE por pelo menos 12 meses antes do início da coleta, ou em acompanhamento por pelo 12 meses antes do início da coleta. Resultados: Etapa 1 - Nove pacientes (graves=3; atenuados=6) com MPS I foram incluídos no estudo, com mediana de idade de diagnóstico de 4,4 anos. Somente o número de cirurgias/ano/paciente foi dependente do tempo de doença (p=0,0004) e da gravidade do fenótipo (p=0,014). Com relação às comparações pré e pós-TRE, as variáveis que apresentaram diferença significativa (média do número/ano/paciente) foram: exames (pré-TRE=10,2+2,7; pós-TRE=22,5+2,1; p=0,005) e internações (pré-TRE=0,05+0,04; pós-TRE=0,30+0,11; p=0,013). Para as demais variáveis, não foi encontrada associação. Etapa 2 - Trinta e quatro pacientes com MPS I (n=12), II (n=17) e VI (n=5) foram incluídos no estudo. Desses, sete não utilizavam TRE (grupo “sem TRE") e 27 faziam uso de tratamento específico (grupo “com TRE"). Não foi encontrada correlação significativa entre tempo de doença e as variáveis estudadas. Considerando a amostra total, foi encontrada diferença entre o grupo “sem TRE” e o grupo “com TRE” em relação à mediana de internações hospitalares e de cirurgias realizadas [1(0-2) vs. 0 (0-1), p=0,015; e 0 (0-2) vs. 0 (0-0), p=0,040, respectivamente]. Para as crianças/adolescentes (<18 anos), não foi encontrada diferença estatística entre os grupos. Os pacientes com comprometimento cognitivo utilizavam mais medicamentos que os demais (p=0,024). Encontrou-se correlação negativa entre as variáveis duração da TRE e número anual de internações (r= -0,504; p=0,007). Discussão/ Conclusões: Este é um dos primeiros estudos a avaliar aspectos relacionados à farmaconomia da TRE para as MPS. De acordo com os resultados obtidos na etapa 2, verifica-se que, desconsiderando-se o custo associado às infusões, o custo do tratamento de pacientes com MPS parece ser menor para aqueles pacientes que utilizam a TRE do que para os pacientes que fazem somente tratamento sintomático. Entretanto, de acordo com a etapa 1 do estudo, a TRE parece não impedir a evolução da doença, pelo menos em relação à MPS I, e, assim, a cada ano de vida do paciente ocorreria um incremento do custo associado ao tratamento. Estudos adicionais, com maior tamanho amostral, deverão ser realizados para confirmar nossos achados. / Introduction: The mucopolysaccharidoses type I (MPS I), II (MPS II) and VI (MPS VI) are lysosomal disorders (LSD) for which enzyme replacement therapy (ERT) with laronidase, idursulfase and galsulfase, respectively, are available. Principal objective: To analyze the annual frequency of medical interventions (number of medical appointments, hospital admissions, surgical procedures, exams performed, medications prescribed, ancillary therapies and the use of medical devices) in a sample of Brazilian patients with MPS I, II and VI, and thus, contribute to the understanding of some pharmacoeconomic aspects related to these diseases. Methodology: Retrospective, exploratory, hospital-based study, based on medical records review, with convenience sampling, which was conducted in two steps (steps 1 and 2). A specific data collection instrument for both steps was designed by the study team, which is multidisciplinary. The chosen outcomes were: annual frequencies of medical interventions (medical appointments, exams, surgical procedures, hospital admissions, medications used and ancillary therapies). Step 1 was a pre-experimental study conducted at the Medical Genetics Service of Hospital de Clínicas de Porto Alegre (SGM-HCPA), and compared the variables of interest between the pre and post-ERT periods for the same group of patients. The patient inclusion criteria were: a biochemical diagnosis of MPS I and regular follow-up at SGM-HCPA since diagnosis; ERT for at least 1 year; no enrollment in any clinical trials involving ERT, and no history of hematopoietic stem cell transplantation. Step 2 was a cross-sectional and multicentric estudy (Centers included: SGM-HCPA), the Department of Medical Genetics of Universidade Estadual de Campinas - UNICAMP, Pontifícia Universidade Católica de Campinas - PUC-Campinas, and the Department of Pediatrics at Universidade Estadual do Rio de Janeiro – UERJ, which compared the variables of interest between different groups of patients (those receiving and those not receiving ERT). For this step only data from 2010 were considered. The inclusion patient criteria were: a biochemical diagnosis of MPS I, II or VI; no enrollment in any clinical trials involving ERT, and no history of hematopoietic stem cell transplantation, to be on ERT for at least 12 months before the start of data collection or to undergo regular follow-up for at least 12 months before the start of data collection. Results: Step 1 – Nine MPS I patients (severe=3; attenuated phenotype=6) were included in the study with median age at diagnosis was 4.4 years. Only the number/year/patient of surgeries was found to be dependent on length of disease (p=0.0004) and on severity of phenotype (p=0.014). Regarding pre- and post-ERT comparisons, the variables for which a significant difference was detected (mean number/year/patient) were exams (pre-ERT, 10.2±2.7; post-ERT, 22.5±2.1; p=0.005) and hospital admissions (pre-ERT, 0.05±0.04; post-ERT, 0.30±0.11; p=0.013). For the other variables, no association was found. Step 2: Thirty-four patients with MPS I, II and VI were included (I=12, II=17, VI=5). From them, 27 on ERT (“ERT group”) and 7 receiving supportive care only (“non-ERT group”). There were no significant correlation between length of disease and any of the variables of interest. There were significant between-group differences in the median number of hospital admissions and surgical procedures, both of which were higher in the non-ERT group [1(0-2) vs. 0 (0-1), p=0,015; e 0 (0-2) vs. 0 (0-0), p=0,040, respectively]. There were no significant between-group differences when only children and adolescents (<18 years) were taken into account. Patients with cognitive involvement used more medications than the others (p=0.024). A correlation was detected between time on ERT and the hospital admissions variable (r= -0.504; p=0.007). Discussion/conclusions: This was one of the first studies to evaluate aspects related to pharmacoeconomics of ERT for MPS. According to the results of step 2, and not acknowledging the costs associated with recombinant enzyme infusions, patients with MPS who undergo ERT generate less cost to SUS than patients on symptomatic treatment. On the other hand, according to the results of step 1, ERT seems not to stop the disease progress, at least in respect to MPS I, and thus, for each year of a patient life occurred an increase in cost associated with treatment. Additional studies with larger sample size are needed to confirm our findings.

Cysteine rich with EGF-like domains 2 (Creld2) is a novel endoplasmic reticulum (ER) resident molecular chaperone that has been recently implicated in the ER stress signalling response (ERSS) and the unfolded protein response (UPR). Global transcriptomic data derived from in vivo mouse models of rare chondrodysplasias; Multiple Epiphyseal Dysplasia (MED Matn3 p.V194D) and Metaphyseal chondrodysplasia type Schmid (MCDS Col10a1 p.N617K), identified a significant upregulation in Creld2 expression in mutant chondrocytes. These chondrodysplasias share a common disease signature consisting of aberrant folding of a matrix component often as a result of inappropriate alignment of intramolecular disulphide bonds. This in turn culminates in toxic protein aggregation, intracellular retention mutant polypeptides and a classical ER stress response. The aim of this study was to further analyse the function of Creld2 in cartilage development and chondrodysplasias in which endochondral bone growth is perturbed. Protein disulphide isomerases (PDIAs) were amongst the most up-regulated genes in the MED and MCDS mouse models, consistent with the prolonged exposure of normally 'buried' cysteine residues. This led to the hypothesis that Creld2 was functioning as a novel PDI-like oxidoreductase to assist in the correct folding and maturation of aggregated misfolded polypeptide chains through REDOX regulated thiol disulphide exchange. A series of Creld2-CXXA substrate trapping mutants were generated in order to determine whether Creld2 possessed inherent isomerase activity. Here potential substrates interacting with Creld2 were 'trapped' as mixed disulphide intermediates, then isolated by immunoprecipitation and identified by mass spectrometry analysis. It was demonstrated that Creld2 possessed a catalytic active CXXC motif in its N-terminus that enabled the molecular chaperone to participate in REDOX regulated thiol disulphide exchange with at least 20 potential substrates including; laminin (alpha3,β3,γ2), thrombospondin 1, integrin alpha3 and type VI collagen. There was also numerous co-chaperones and foldases thought to be part of a specialised protein-protein interactome (PPI) for folding nascent polypeptides translocating the ER lumen. Moreover, co-immunoprecipitation experiments supported a protein-protein interaction between Creld2 and mutant matrilin-3, thereby inferring a potential chondro-protective role in resolving non-native disulphide bonded aggregates in MED. An established biochemical approach was employed to test the hypothesis that all MATN3-MED disease causing mutations have a generic cellular response to the β-sheet V194D mutation, consisting of intracellular retention, protein aggregation and ER stress induction. Several missense mutations were selected for analyses which encompassed a spectrum of disease severity and included examples of both β-sheet and alpha helical mutations. It was possible to define a reliable and reproducible assay for categorising MATN3 missense mutations into pathological or benign based on these basic parameters. This study was extended further to determine whether there were common pathological mechanisms behind MED and Bethlem myopathy (BM) caused by missense mutations in von Willebrand Factor A domain (vWF-A) containing proteins (matrilin-3 and type VI collagen respectively). We chose to compare and contrast the effects of an archetypal MATN3-MED causing mutation (R121W) with the equivalent COL6A2-BM causing mutation (R876H). These mutations compromised protein folding and maturation, resulting in the familiar disease profile of intracellular retention, protein aggregation and an ER stress response in an artificial overexpression system. However, the mutant C2 domain was efficiently targeted for degradation whilst mutant matrilin-3 vWF-A domain appeared to be resistant to these molecular processes.Molecular genetics was employed to study the role of Creld2 in vivo. Creld2-/- null mice (both global and conditional) were generated to directly examine the role of Creld2 in endochondral bone growth. Global knock-out mice were viable with no overt phenotype at birth. However, female Creld2-/- null mice showed a significant reduction in body weight and tibia bone length at 3 weeks of age. A cartilage specific knock-out was generated to determine whether these skeletal abnormalities were attributed to a systemic or a direct effect on cartilage development. [Creld2Flox/Flox Col2Cre (+)] demonstrated a severe chondrodysplasia with significantly reduced body weight and long bone growth compared to control littermates. Morphological and histochemical analysis of mutant growth plates revealed gross disorganisation of the chondrocyte columns with extensive regions of hypocellularity. These pathological features were confirmed to be the result of reduced chondrocyte proliferation and increased/spatially dysregulated apoptosis throughout all zones of differentiation. Taken together, these data provide evidence that Creld2 possesses isomerase activity and exhibits distinct substrate specificity. Furthermore, Creld2 has a fundamental role in post-natal cartilage development and chondrocyte differentiation in the growth plate.

O presente trabalho apresenta o estudo da esteira de turbinas eólicas de eixo horizontal, utilizando um modelo de turbina eólica testada em túnel de vento. Trata-se de uma turbina eólica de duas pás com 10 m de diâmetro, conhecida como UAE Phase VI, testada pelo NREL no túnel de vento NASA Ames. Desta turbina foi obtida a geometria e dados experimentais, como o coeficiente de empuxo, além das características do túnel de vento. Utilizando este experimento como modelo, são realizadas simulações com equações médias de Reynolds e modelo de turbulência k-ω SST, com o uso de malha móvel e abordagens permanente e transiente, com o software de volumes finitos Star-CCM+®. São empregadas malhas poliédricas em um domínio de grande extensão na esteira, para apenas uma velocidade do vento. São apresentados três modelos de esteira, dois analíticos e outro considerado numérico, para fins de comparação com os resultados simulados. Estes modelos de esteira tem o objetivo de descrever o campo de velocidade a jusante da turbina. Os dados de entrada dos modelos são os mesmos, retirados da simulação. Além dos modelos para o déficit de velocidade, são apresentados equacionamentos para se obter a intensidade de turbulência na esteira, com bons resultados. No modelo analítico de esteira PARK, a constante de decaimento é baixa, pois o caso estudado possui escoamento sem grandes perturbações. Verifica-se que esta constante deve variar ao longo da esteira para se ajustar ao campo médio de velocidades simulado. Outro modelo analítico proposto por Werle foi estudado, não apresentando boa concordância com a simulação, pois apresenta um rápido incremento da velocidade na linha central na esteira distante. Este modelo se ajustou bem com o campo não perturbado a montante da turbina. O modelo de viscosidade turbulenta é um modelo de esteira com equacionamento original de segunda ordem para obter um campo de velocidade. Contudo, é apresentado e calculado com uma simplificação para primeira ordem, obtendo-se somente a velocidade na linha de centro. Este modelo tem o melhor ajuste com a simulação. A metodologia adotada para simulação numérica do escoamento apresenta boa concordância com os resultados experimentais de CT, já os dados anemométricos medidos na esteira próxima não foram reproduzidos com a mesma qualidade, contudo aproximam-se de forma qualitativa. A comparação com os modelos de esteira da literatura confirma que o modelo de viscosidade turbulenta é o que mais se aproxima dos resultados da simulação. / This study presents wakes of horizontal axis wind turbines, using as model a wind turbine tested in wind tunnel. It is a two bladed wind turbine with 10 m diameter, known as UAE Phase VI, tested by NREL in the NASA Ames wind tunnel. From this experiment was obtained the geometry and measured data, as the thrust coefficient, and the characteristics of the wind tunnel. Using this experiment as model, simulations are performed with Reynolds Averaged Navier-Stokes equations and k-ω SST turbulence model, using moving mesh and permanent and transient approaches, with help of the finite volume software Star-CCM+®. Polyhedral meshes are employed in a domain with large extent downwind, for just one wind speed. There are presented three wake models, two analytical and other considered numerical, for purposes of comparison with simulated results. These wake models aims to describe the velocity field downstream of the turbine. The input data of the models are the outputs of the simulations. Besides the models for the velocity deficit, are given equations to derive the turbulence intensity on the wake, with good results. In the analytical PARK wake model, the decay constant is low, as the study case has low perturbed flow. It is noted that this constant should vary along the wake to fit the average speeds field simulated. Another analytical model proposed by Werle is presented, not showing good results when comparing with the simulation, since it shows a rapid increase in the centerline velocity on the far wake. This model fits well with the undisturbed field upwind of the turbine. The turbulent viscosity is a wake model with the original second order equation to obtain the velocity field. However, it is presented and calculated in a simplified way to first order, obtaining only the centerline velocity. This model has the best fit with the simulation. The methodology adopted for numerical simulation of the flow shows good agreement with the experimental results of CT, since the wind data measured on the near wake are not reproduced with the same quality, yet close to a qualitative manner. The comparison with the wake models from the literature confirms that the eddy viscosity wake model is closest to the simulation results.

L’objectif de ce travail est d’élaborer des méthodes peu chères pour produire desmatériaux semi-conducteurs pouvant entrer dans la fabrication de cellules solaires de type"eta" (extremely thin absorber). Ces cellules sont constituées d’une couche extrêmement fined’un adsorbeur inorganique dont la bande interdite est situé entre 1.1 et 1.8 eV placée entredeux nanostructures transparentes l’une de type n et l’autre de type p et dont les bandesinterdites doivent être supérieurs à 3.3 eV. Une couche compacte et des nanofils de ZnO ontété préparés en mode galvanostatique. Les dimensions des nanofils ont été contrôlées à l’aidede la couche compacte et de la densité du courant appliqué. La photosensibilisation desnanofils par des couches uniformes de CdS, CdSe et CdTe préparée par la méthode SILAR(Successive Ionic Layer Adsorption and Reaction) a été étudiée. Les propriétés de cescouches ont été améliorées par recuit et traitement chimique. En ce qui concerne les finescoquilles de CdTe deux autres méthodes de sensibilisation ont été également étudiées : la CSS(Close Spaced Sublimation) et les QDs (Quantum Dots). La première méthode conduit à unfaible recouvrement alors que la seconde produit un matériau mal défini optiquement. Leshétérostructures formées sur les nanofils ont été complétées par une couche de CuSCN, unsemi-conducteur de type p, préparée par trois méthodes différentes. L’influence de lamorphologie de ces couches sur les propriétés des cellules eta a été étudiée. Les filmspréparés par électrodéposition et SILAR sont plus rugueux que ceux obtenus parimprégnation et leur conductivité est moins bonne. Les hétérostructures (avec CdS et CdSecomme absorbeurs) ont été testées dans une cellule photoélectrochimique et les rendementsobtenus (jusque 2%) montrent une amélioration certaine des propriétés de ces matériauxpréparée par SILAR-modifiée ainsi que des interfaces ZnO/absorbeur. La qualité desmatériaux obtenus par SILAR montre qu’aujourd’hui on peut s’attendre à une Renaissance decette technique. / The development of semiconducting materials for the extremely thin absorber (eta)solar cell using cheap and scalable methods was the main objective of this work. The eta-solarcell is composed of all inorganic materials consisting of an extremely thin layer of absorbingmaterial (1.1 <Eg< 1.8 eV) sandwiched between nanostructured transparent electron and holeconductors (Eg ≥ 3.3 eV). Compact and defect free ZnO thin film and nanowires (NWs) wereprepared galvanostatically. The ZnO nanowire dimensions were controlled with the ZnO seedlayer or the applied current density. The photosensitization of the ZnO nanowires withconformal layers of CdS, CdSe and CdTe prepared by Successive Ionic Layer Adsorption andReaction (SILAR) was studied. The improvement of the absorber structural and opticalproperties by annealing and chemical treatment was achieved. The Close Spaced Sublimation(CSS) and Quantum Dot (QD) sensitization were also used for CdTe thin shell deposition,while the first method produced low coverage, the second resulted in better coverage but withnot optimal optical features. The ZnO NW/absorber heterostructure was completed with ahole conducting CuSCN layer. The influence of the CuSCN layer (prepared by three methods)morphology on the eta-solar cell performance is discussed. Electrodeposited and SILARprepared films exhibited rougher surfaces than that by the Impregnation technique (whichaffects the electrical conductivity). The ZnO/absorber core/shell heterostructures were alsotested in a photoelectrochemical cell. The recorded efficiencies (up to 2 %) for the case ofCdS and CdSe photosensitizers demonstrated an improvement of the ZnO/absorber interfacesand the material quality achieved by the modified-SILAR technique. These results let us toconsider that today a Renascence of the SILAR method is happening.

Made available in DSpace on 2014-12-17T13:53:33Z (GMT). No. of bitstreams: 1 LuizCSJ_DISSERT.pdf: 1251184 bytes, checksum: 11adb0eaeb2b09775607dded39ab4867 (MD5) Previous issue date: 2012-08-27 / The objective is to analyze the relationship between risk and number of stocks of a portfolio for an individual investor when stocks are chosen by "naive strategy". For this, we carried out an experiment in which individuals select actions to reproduce this relationship. 126 participants were informed that the risk of first choice would be an asset average of all standard deviations of the portfolios consist of a single asset, and the same procedure should be used for portfolios composed of two, three and so on, up to 30 actions . They selected the assets they want in their portfolios without the support of a financial analysis. For comparison we also tested a hypothetical simulation of 126 investors who selected shares the same universe, through a random number generator. Thus, each real participant is compensated for random hypothetical investor facing the same opportunity. Patterns were observed in the portfolios of individual participants, characterizing the curves for the components of the samples. Because these groupings are somewhat arbitrary, it was used a more objective measure of behavior: a simple linear regression for each participant, in order to predict the variance of the portfolio depending on the number of assets. In addition, we conducted a pooled regression on all observations by analyzing cross-section. The result of pattern occurs on average but not for most individuals, many of which effectively "de-diversify" when adding seemingly random bonds. Furthermore, the results are slightly worse using a random number generator. This finding challenges the belief that only a small number of titles is necessary for diversification and shows that there is only applicable to a large sample. The implications are important since many individual investors holding few stocks in their portfolios / Objetiva-se neste trabalho analisar a rela??o entre o risco e o n?mero de a??es de uma carteira para um investidor individual quando as a??es s?o escolhidas atrav?s da estrat?gia ing?nua . Para isso, realizou-se um experimento em que indiv?duos selecionam a??es para reproduzir essa rela??o. 126 participantes foram informados de que o risco do primeiro ativo escolhido seria uma m?dia entre todos os desvios padr?es das carteiras compostas por um ?nico ativo, e o mesmo procedimento deveria ser usado para as carteiras compostas por duas, tr?s e assim por diante, at? 30 a??es. Eles selecionaram os ativos que queriam em suas carteiras sem o suporte de uma an?lise financeira. Para fins de compara??o, tamb?m foi realizada uma simula??o hipot?tica de 126 investidores que selecionaram a??es de um mesmo universo, atrav?s de um gerador de n?meros aleat?rios. Assim, cada participante real pode ser comparado por um investidor hipot?tico aleat?rio frente a mesma oportunidade. Foram observados padr?es nas carteiras dos participantes individuais, caracterizando as curvas para as componentes das amostras. Como esses agrupamentos s?o subjetivos, utilizou-se posteriormente uma medida mais objetiva de comportamento: uma regress?o linear simples para cada participante, com o intuito de predizer a vari?ncia da carteira em fun??o do inverso do n?mero de ativos. Al?m disso, foi realizada uma regress?o agrupada sobre todas as observa??es, atrav?s de an?lise cross-section. A rela??o exponencial negativa ocorre, em m?dia, mas n?o para a maioria dos indiv?duos, muitos dos quais efetivamente desdiversificam quando adicionam t?tulos aparentemente aleat?rios. Os resultados alcan?ados s?o ligeiramente piores usando-se um gerador de n?mero aleat?rio. Esta descoberta desafia a cren?a de que apenas um pequeno n?mero de t?tulos ? necess?rio para que haja diversifica??o e mostra que ? aplic?vel apenas a uma amostra grande. As implica??es s?o importantes uma vez que muitos investidores individuais mantem poucas a??es em suas carteiras

Introdução: As mucopolisaccaridoses tipo I (MPS I), tipo II (MPS II) e tipo VI (MPS VI) são doenças lisossômicas (DL) para as quais está disponível a terapia de reposição enzimática (TRE) com laronidase, idursulfase e galsufase, respectivamente. Objetivo Primário: Analisar a frequência anual de intervenções médicas (número de consultas, internações, cirurgias, exames solicitados, medicamentos prescritos, equipamentos de uso crônico e outras formas de terapia) em uma amostra de pacientes brasileiros com MPS I, II e VI e, desta forma, contribuir para o conhecimento dos aspectos farmacoeconômicos relacionados a essas doenças. Metodologia: Estudo exploratório, retrospectivo, de base hospitalar, baseado em revisão de prontuário, com amostragem por conveniência, e que foi realizado em duas etapas (etapas 1 e 2). Um instrumento específico para a coleta de dados de ambas as etapas foi construído pela equipe do estudo, que é multidisciplinar. Os desfechos de interesse foram as frequências anuais de intervenções médicas (consultas, exames, cirurgias, internações, medicamentos utilizados, outras formas de terapia). A etapa 1 consistiu em estudo pré-experimental, realizado no Serviço de Genética Médica do Hospital de Clínicas de Porto Alegre (SGM-HCPA), e que comparou as variáveis de interesse, para o mesmo grupo de pacientes, entre o período pré e pós-TRE. Os critérios de inclusão dessa etapa foram: ter diagnóstico confirmado de MPS I; estar em acompanhamento regular no SGM-HCPA desde o diagnóstico; estar em TRE por pelo menos um ano; e não ter participado de ensaio clínico envolvendo TRE ou ter realizado transplante de células-tronco hematopoiéticas. A etapa 2 foi transversal, multicêntrica (centros incluídos: SGMHCPA, Departamento de Genética Médica da Universidade Estadual de Campinas - UNICAMP, Pontifícia Universidade Católica de Campinas – PUC-Campinas, e Departamento de Pediatria da Universidade Estadual do Rio de Janeiro - UERJ), e comparou as variáveis de interesse entre grupos diferentes de pacientes (aqueles recebendo TRE e aqueles não recebendo TRE). Para essa etapa, foram considerados somente os dados relativos a 2010, sendo os seguintes os critérios de inclusão dos pacientes: ter diagnóstico confirmado de MPS I, II e VI; não estar participando de nenhum ensaio clínico envolvendo TRE ou ter realizado transplante de células-tronco hematopoiéticas; estar em TRE por pelo menos 12 meses antes do início da coleta, ou em acompanhamento por pelo 12 meses antes do início da coleta. Resultados: Etapa 1 - Nove pacientes (graves=3; atenuados=6) com MPS I foram incluídos no estudo, com mediana de idade de diagnóstico de 4,4 anos. Somente o número de cirurgias/ano/paciente foi dependente do tempo de doença (p=0,0004) e da gravidade do fenótipo (p=0,014). Com relação às comparações pré e pós-TRE, as variáveis que apresentaram diferença significativa (média do número/ano/paciente) foram: exames (pré-TRE=10,2+2,7; pós-TRE=22,5+2,1; p=0,005) e internações (pré-TRE=0,05+0,04; pós-TRE=0,30+0,11; p=0,013). Para as demais variáveis, não foi encontrada associação. Etapa 2 - Trinta e quatro pacientes com MPS I (n=12), II (n=17) e VI (n=5) foram incluídos no estudo. Desses, sete não utilizavam TRE (grupo “sem TRE") e 27 faziam uso de tratamento específico (grupo “com TRE"). Não foi encontrada correlação significativa entre tempo de doença e as variáveis estudadas. Considerando a amostra total, foi encontrada diferença entre o grupo “sem TRE” e o grupo “com TRE” em relação à mediana de internações hospitalares e de cirurgias realizadas [1(0-2) vs. 0 (0-1), p=0,015; e 0 (0-2) vs. 0 (0-0), p=0,040, respectivamente]. Para as crianças/adolescentes (<18 anos), não foi encontrada diferença estatística entre os grupos. Os pacientes com comprometimento cognitivo utilizavam mais medicamentos que os demais (p=0,024). Encontrou-se correlação negativa entre as variáveis duração da TRE e número anual de internações (r= -0,504; p=0,007). Discussão/ Conclusões: Este é um dos primeiros estudos a avaliar aspectos relacionados à farmaconomia da TRE para as MPS. De acordo com os resultados obtidos na etapa 2, verifica-se que, desconsiderando-se o custo associado às infusões, o custo do tratamento de pacientes com MPS parece ser menor para aqueles pacientes que utilizam a TRE do que para os pacientes que fazem somente tratamento sintomático. Entretanto, de acordo com a etapa 1 do estudo, a TRE parece não impedir a evolução da doença, pelo menos em relação à MPS I, e, assim, a cada ano de vida do paciente ocorreria um incremento do custo associado ao tratamento. Estudos adicionais, com maior tamanho amostral, deverão ser realizados para confirmar nossos achados. / Introduction: The mucopolysaccharidoses type I (MPS I), II (MPS II) and VI (MPS VI) are lysosomal disorders (LSD) for which enzyme replacement therapy (ERT) with laronidase, idursulfase and galsulfase, respectively, are available. Principal objective: To analyze the annual frequency of medical interventions (number of medical appointments, hospital admissions, surgical procedures, exams performed, medications prescribed, ancillary therapies and the use of medical devices) in a sample of Brazilian patients with MPS I, II and VI, and thus, contribute to the understanding of some pharmacoeconomic aspects related to these diseases. Methodology: Retrospective, exploratory, hospital-based study, based on medical records review, with convenience sampling, which was conducted in two steps (steps 1 and 2). A specific data collection instrument for both steps was designed by the study team, which is multidisciplinary. The chosen outcomes were: annual frequencies of medical interventions (medical appointments, exams, surgical procedures, hospital admissions, medications used and ancillary therapies). Step 1 was a pre-experimental study conducted at the Medical Genetics Service of Hospital de Clínicas de Porto Alegre (SGM-HCPA), and compared the variables of interest between the pre and post-ERT periods for the same group of patients. The patient inclusion criteria were: a biochemical diagnosis of MPS I and regular follow-up at SGM-HCPA since diagnosis; ERT for at least 1 year; no enrollment in any clinical trials involving ERT, and no history of hematopoietic stem cell transplantation. Step 2 was a cross-sectional and multicentric estudy (Centers included: SGM-HCPA), the Department of Medical Genetics of Universidade Estadual de Campinas - UNICAMP, Pontifícia Universidade Católica de Campinas - PUC-Campinas, and the Department of Pediatrics at Universidade Estadual do Rio de Janeiro – UERJ, which compared the variables of interest between different groups of patients (those receiving and those not receiving ERT). For this step only data from 2010 were considered. The inclusion patient criteria were: a biochemical diagnosis of MPS I, II or VI; no enrollment in any clinical trials involving ERT, and no history of hematopoietic stem cell transplantation, to be on ERT for at least 12 months before the start of data collection or to undergo regular follow-up for at least 12 months before the start of data collection. Results: Step 1 – Nine MPS I patients (severe=3; attenuated phenotype=6) were included in the study with median age at diagnosis was 4.4 years. Only the number/year/patient of surgeries was found to be dependent on length of disease (p=0.0004) and on severity of phenotype (p=0.014). Regarding pre- and post-ERT comparisons, the variables for which a significant difference was detected (mean number/year/patient) were exams (pre-ERT, 10.2±2.7; post-ERT, 22.5±2.1; p=0.005) and hospital admissions (pre-ERT, 0.05±0.04; post-ERT, 0.30±0.11; p=0.013). For the other variables, no association was found. Step 2: Thirty-four patients with MPS I, II and VI were included (I=12, II=17, VI=5). From them, 27 on ERT (“ERT group”) and 7 receiving supportive care only (“non-ERT group”). There were no significant correlation between length of disease and any of the variables of interest. There were significant between-group differences in the median number of hospital admissions and surgical procedures, both of which were higher in the non-ERT group [1(0-2) vs. 0 (0-1), p=0,015; e 0 (0-2) vs. 0 (0-0), p=0,040, respectively]. There were no significant between-group differences when only children and adolescents (<18 years) were taken into account. Patients with cognitive involvement used more medications than the others (p=0.024). A correlation was detected between time on ERT and the hospital admissions variable (r= -0.504; p=0.007). Discussion/conclusions: This was one of the first studies to evaluate aspects related to pharmacoeconomics of ERT for MPS. According to the results of step 2, and not acknowledging the costs associated with recombinant enzyme infusions, patients with MPS who undergo ERT generate less cost to SUS than patients on symptomatic treatment. On the other hand, according to the results of step 1, ERT seems not to stop the disease progress, at least in respect to MPS I, and thus, for each year of a patient life occurred an increase in cost associated with treatment. Additional studies with larger sample size are needed to confirm our findings.

This Master’s thesis is one of two parts of a combined project called Sustainable development around the Lake Victoria, the purpose of which is to investigate the importance of local anchoring and active participation in the work towards sustainable development. The present study aims to investigate whether men and women are empowered by the participatory rural appraisal (PRA) process promoted in their villages by the VI Agroforestry Project Masaka/Rakai. The study is primarily based on interviews with farmers within the project area and brings up a local perspective on the concepts sustainable development, active participation and empowerment. The study shows that to improve the farmers’ livelihood and to create a sustainable development in the community, the farmers have to be actively involved in, and in control of, the process. The people concerned are the ones who are most familiar with the local society and to make the development sustainable in a long-term perspective, activities and solutions need to be adapted to local conditions and circumstances. However, the farmers must first know what kind of development they want, i.e. where they want to go, and be aware of what means they may utilize to get there. In other words, the farmers need to be empowered before they can be in charge of their development process. According to the interviews, the farmers are encouraged by the VI Agroforestry Project’s PRA process to discuss their present situation with each other, to develop strategies of how to improve it and to increase the collaboration within the villages. From this I come to the conclusion that the PRA process promoted in their villages has made the farmers more aware of their situation and better equipped to identify opportunities in their neighbourhood. Therefore, I argue that the farmers are empowered by the VI Agroforestry Project’s PRA process. The study is published both as a Master’s thesis for the Environmental Science Programme, Linköping University and as a Minor Field Study for Sida. There are only editorial differences between the two versions.