Recent years have witnessed significant research into the acute effects of exercise on appetite, energy intake and gut hormone responses. The experiments in this thesis have further investigated this topic by examining the appetite, acylated ghrelin, peptide YY and energy intake responses to energy deficits induced via different exercise protocols and food restriction. To achieve this, 48 young healthy males (mean (SD): age 23 (3) years, body mass index 23.7 (2.7) kg.m-2, maximum oxygen uptake 52.9 (9.8) mL.kg 1.min-1) were recruited into four studies. In study one, 60 min of treadmill running at 70% of VO2 max did not stimulate any increases in appetite or daily energy intake regardless of whether the exercise was performed after breakfast or in the fasted state. In study two, six 30 s Wingate tests stimulated increases in appetite during the subsequent hours compared with 60 min of cycling at 68% of VO2 max. Differences in appetite appeared to be unrelated to changes in plasma acylated ghrelin concentrations and did not influence ad libitum energy intake. Subsequently, endurance exercise resulted in a significantly greater negative daily energy balance than sprint exercise due to a larger exercise energy expenditure. Study three revealed that appetite and energy intake did not differ from a resting control trial after either ten, 4 min cycling bouts at 85 90% of VO2 max separated by 2 min of rest or 60 min of constant cycling at 60% of VO2 max. This occurred despite elevated PYY3-36 concentrations during the hours after exercise. Finally, study four showed that an energy deficit of ~1475 kJ stimulated increases in appetite when induced via food restriction but not when achieved by an acute bout of exercise. This was associated with differences in plasma PYY3-36 concentrations but did not appear to be related to changes in circulating levels of acylated ghrelin and did not influence energy intake. This thesis has shown that appetite perceptions do not differ from a resting control trial during the hours after continuous endurance exercise. Alternatively, supramaximal cycling exercise and subtle reductions in food intake stimulated increases in appetite during the subsequent hours. Such increases in appetite do not appear to be related to changes in acylated ghrelin but may be influenced by plasma PYY3-36 concentrations. Despite differences in appetite, daily energy intake was unaffected by all interventions.

A telenfermagem é a utilização dos recursos tecnológicos e dos sistemas de comunicação em prol do desenvolvimento da enfermagem. Vários países utilizam a telenfermagem na gestão de cuidados de saúde com resultados positivos. Na Enfermagem brasileira este é um campo a ser explorado, assim o objetivo desse trabalho foi de desenvolver estratégias de telenfermagem no atendimento a pacientes com bexiga neurogênica, usuários de cateterismo urinário intermitente limpo, atendidos em um centro de reabilitação de um hospital universitário. O estudo foi realizado em 3 etapas: etapa 1 - revisão integrativa da literatura para investigar na literatura a aplicação da telenfermagem; etapa 2 - elaboração e validação do protótipo de manual de telenfermagem para subsidiar o enfermeiro na implantação da intervenção de telenfermagem no atendimento ao paciente com bexiga neurogênica, usuário de cateterismo urinário intermitente limpo; etapa 3: estudo piloto sobre a implantação de intervenção de telenfermagem no atendimento ao paciente em uso de cateterismo urinário intermitente limpo. O manual de telenfermagem foi construído procurando contextualizar a telenfermagem no Brasil e no exterior, com seleção da teoria de Orem para direcionar o teleatendimento ao paciente, e um referencial sobre os cuidados para a realização de um teleatendimento. Descrição de recursos tecnológicos disponíveis que podem ser utilizados no teleatendimento, além de informações sobre os cuidados de saúde ao paciente com bexiga neurogênica e na realização do cateterismo urinário intermitente limpo. O manual foi validado em aparência e conteúdo por peritos com auxílio de um instrumento de avaliação. A intervenção de telenfermagem mostrou resultados importantes como forma de implementação do tratamento de saúde tradicional ao paciente, bem como evidenciou barreiras que precisam ser superadas para que esse tipo de atendimento seja realizado, como uma pesquisa efetiva com possibilidades de implementação posterior ao serviço de saúde. Os dados foram analisados através de estatística descritiva (frequência e porcentagem) com auxílio do programa SPSS (Statistical Package for Social Science), versão 15.0. O nível de concordância entre os peritos foi considerado de 70% para cada aspecto do instrumento utilizado / Telenursing is the use of technological resources and communication systems for the development of nursing. A great number of countries employ telenursing on healthcare management with positive results, however, it is a field yet to be explored in Brazilian nursing. The aim of this study was to develop telenursing strategies in the care to patients with neurogenic bladder, users of clean intermittent urinary catheterization, treated in a university hospital rehabilitation center. The study was carried out in 3 stages: stage 1 - integrative literature review to research the application of telenursing; stage 2 - development and validation of a telenursing booklet prototype to support nurses in implementing telenursing intervention on care to patients with neurogenic bladder, users of clean intermittent urinary catheterization; stage 3 - pilot study on the application of telenursing intervention in care to patients using clean intermittent urinary catheterization. The telenursing booklet was developed to contextualize telenursing in Brazil and abroad, selecting Orem\'s theory to guide the telecare to the patient and a framework about the care needed to carry out telecare. Description of available technological resources that can be used in telecare, as well as information about healthcare to patients with neurogenic bladder and in the procedure of clean intermittent urinary catheterization. The booklet underwent face and content validation by experts with aid from an assessment instrument. Telenursing intervention showed important results as a way to implement the traditional health treatment to the patient, also pointing out barriers that must be overcome in order to perform this kind of treatment, such as an effective research with possibilities of subsequent implementation at the health service. Data were analyzed through descriptive statistics (frequency and percentage) using SPSS (Statistical Package for Social Science) software version 15.0. The level of concordance considered among the experts was of 70% for each aspect of the used instrument

O arroz é uma das principais culturas irrigadas no mundo e com as maiores quantidades de água aplicada. No estado de Santa Fe, Argentina, o arroz é a principal cultura irrigada, com eficiências de irrigação muito baixas (25 a 40%). O método tradicional de inundação contínua (Ic) não aproveita as precipitações pluviais, que representam entre 30 a 40% da evapotranspiração real (ETr). Foram desenvolvidos experimentos em diferentes agro-ecossistemas em San Javier (Santa Fe), objetivando avaliar o arroz com aspersão (AS), inundação intermitente (In) e Ic, visando aumentar as eficiências de irrigação e de uso da água. Na safra 2011-12 predominou Planossolo nátrico (PlaNa), altamente adensado e, na safra 2012-13, Regossolo abrupto (RegAb), com boa drenagem. A variedade de arroz utilizada foi Puitá INTA CL, índica e anaeróbica. A aplicação do Nitrogênio (2011-2012) foi feita de duas maneiras: 20% na semeadura e o restante no perfilhamento, segundo manejo habitual dos produtores (adubação tradicional, AT), e a maneira alternativa, parcelando a adubação (AP). Na safra 2012-2013 foram avaliadas diferentes doses de adubação: T0, testemunha; T1 e T2, com 20 e 40% do requerimento total do N respectivamente. O delineamento estatístico em 2011-12 foi parcelas divididas e fatorial em 2012-13. Em Ic foi mantida uma altura de água constante (6 a 8 cm) e, em In, manteve-se sempre o solo PlaNa saturado e RegAb com potencial mátrico de -10 kPa. Em AS, no PlaNa utilizou-se como controle -25 kPa mudando para -10 kPa no RegAb. Foi realizado um balanço hídrico (BH) determinando, entre outras componentes, lâmina de irrigação bruta (Lib); ETr e percolação profunda (PerIr). Foi observada a fenologia, avaliada as taxas de crescimento e índice de área foliar. Na colheita foi determinada a produtividade (PG), seus componentes e concentrações de N no tecido vegetal. Foram determinadas as eficiências de irrigação, de uso de água, água virtual e pegada hídrica. Em 2011-12 a PG foi semelhante nos manejos Ic e In (média de 9,8 Mg ha-1), com redução de 24% no AS devido a forte estresse hídrico. No PlaNa a taxa de percolação foi 2,8 mm d-1, resultando a Lib e PerIr dos manejos In e Ic quase iguais, de 6.300 e 1.540 m3 ha-1 respectivamente. Em 2012- 13 a PG foi similar em todos os manejos da água (média de 10 Mg ha-1), com resposta significativa às doses de adubação. A taxa de percolação do RegAb triplicou a PlaNa, pelo que a Lib e PerIr dos manejos Ic, In e AS apresentaram grandes diferenças: 11.600, 9.120 e 5.320 m3 ha-1 para Lib e 8.580, 6.190 e 1.980 m3 ha-1 para PerIr. A biomassa aérea teve comportamento contrastante, com crescimento sigmóide em 2011-12, embora linear em 2012-13. A pegada hídrica da produção de arroz em San Javier foi de 261,7 Mm3 ano-1, representando a água azul, verde e cinza 52, 41 e 7%, respectivamente. In apresentou-se como o manejo mais versátil, sendo sua utilização promissora, no entanto AS pode ser utilizado em solos com boa drenagem. Nestes solos seria mais apropriada a adubação parcelada. / Rice is one of the main crops under irrigation in the world, with the highest amount of water applied. In Santa Fe, Argentina, rice is the main crop under irrigation. The traditional method of continuous flooding (CF) does not use rainfall (R), which represents 30 to 40% of the actual evapotranspiration (ETa). Experiments were conducted in different agroecosystems in San Javier, Santa Fe, with the aim of assessing rice cultivation with sprinkle irrigation (SI), intermittent flooding (IF), and CF to increase irrigation and water use efficiencies. In 2011-2012, the predominant soil was typic Natraqualf (Nat), highly compacted, and in 2012-2013 it was Orthent Abruptic (OrtAb), with good drainage. The rice variety used was lowland, Puitá INTA CL. 20% of the fertilizer with N (2011-2012) was applied at sowing and the rest at tillering stage, according to the farmers\' usual management (traditional fertilization T) and an alternative management, thus resulting in partial fertilization (PF). In 2012- 2013, different doses of N were evaluated: T0, no fertilizer; T1 and T2, with 20 and 40% of the total N requirement, respectively. In 2011-2012, the statistical design consisted of split plots, while in 2012-2013 the design was a factorial one. In CF ponded water was maintained at a constant height of 6-8 cm during the whole cycle, whereas in IF Nat was always saturated or with -10 kPa in the case of OrtAb. In SI treatments, -25 kPa was used as control in Nat, changing to -10 kPa in OrtAb. A water balance (WB) was made, determining, among other components, gross irrigation depth (GId), ETa, and deep percolation arising from irrigation (DPir). Phenology was observed, and growth rates and leaf area index were evaluated. Yields (Y, 14%), its components, and the concentrations of nitrogen in plant tissues were determined at harvest time. The efficiencies of irrigation, water use, and water footprint were determined. In 2011-2012, Y was similar in CF and IF managements (average of 9.8 Mg ha-1), with a 24% reduction in SI due to strong water stress. Nat had a percolation of 2.8 mm day-1, thus GId and DPir in IF and CF managements were very similar -- 6,300 and 1,540 m3 ha-1, respectively. In 2012-2013, Y was similar in all water managements (average of 10 Mg ha-1), with a significant response to fertilization rates. The percolation of OrtAb tripled that of Nat, so GId and DPir in CF, IF, and SI systems had significant differences: 11,600, 9,120, and 5,320 m3 ha-1 for GId; and 8,580, 6,190, and 1,980 m3 ha-1 for DPir. The aboveground dry weight had a contrasting behavior, with a sigmoidal growth in 2011-2012 and a linear growth in 2012-2013. The water footprint of rice production in San Javier was 261.7 Mm3 year-1 -- blue, green, and gray water representing 52, 41, and 7%, respectively. IF management was the most versatile, being a promising technique to be used in the field, while SI can be used in soils with good drainage. In these soils, splitting fertilization would be more appropriate.

Essa pesquisa teve como objetivo principal avaliar o desempenho de um reator de leito estruturado e fluxo contínuo, sob diferentes condições operacionais como aeração intermitente e recirculação do efluente, na remoção biológica de carbono e nitrogênio de uma água residuária sintética. Foi utilizado um reator vertical, construído em acrílico, com um volume total de 11,6 L e um volume útil de 6,1 L. O meio suporte utilizado para fixação dos microrganismos foi espuma de poliuretano na forma de cilindros dispostos verticalmente suportados por hastes, presos nas extremidades. A razão de recirculação utilizada durante todo o experimento foi igual a 5. Os períodos adotados de aeração e não aeração foram de 2 horas e 1 hora, respectivamente. Estabelecidos a vazão de recirculação e os períodos de aeração e não aeração, estudou-se o efeito da variação do TDH na eficiência de remoção de \'N\'-total e DQO. Como resultado, observou-se que a melhor condição operacional foi com TDH de 12 horas, que apresentou eficiência de remoção de \'N\'-total de 82% e remoção de DQO de 89%. O sistema mostrou-se estável ao ser operado nessas condições, apresentando pouca variação nos resultados. Por outro lado, quando o sistema foi operado com TDH de 8 horas e 10 horas, houve queda nas eficiências de remoção de \'N\'-total para valores de 49% e 45% respectivamente. Com relação à remoção de DQO, as eficiências com TDH de 8 horas e 10 horas foram de 85% e 88% respectivamente, indicando uma boa remoção de matéria orgânica mesmo com TDH menor. Não houve presença significativa de biomassa no efluente, indicando que a biomassa permaneceu fortemente aderida ao meio suporte. Foram realizados ensaios cinéticos para determinação das velocidades de nitrificação e desnitrificação da biomassa do reator operando em sua melhor condição operacional. Por meio e perfis temporais em reatores em batelada, obteve-se uma velocidade de nitrificação via \'N\'-amoniacal de 1,43 mg\'N\'/gSSV.h. As velocidades de nitrificação via \'N\'-nitrito foram 1,87 mg\'N\'/gSSV.h e 1,3 mg\'N\'/gSSV.h. Na desnitrificação de \'N\'-nitrato à \'N\'-nitrito, foram obtidas velocidades de 3,4 mg\'N\'/gSSV.h e 4,0 mg\'N\'/gSSV.h. Para a desnitrificação de \'N\'-nitrito a \'N IND.2\', as velocidades obtidas foram 2,3 mg\'N\'/gSSV.h e 2,9 mg\'N\'/gSSV.h. / This research aimed at evaluating the performance of a structured bed reactor continuously fed, operated with intermittent aeration and effluent recirculation, in the biological removal of carbon and nitrogen from a synthetic effluent. A vertical reactor was built in acrylic, with a total volume of 11.6 L and a working volume of 6.1 L. Polyurethane foam cylinders supported by sticks vertically disposed were used for biomass attachment. The recirculation flow rate was 5. Aerated and non-aerated periods ware 2 hours and 1 hour, respectively. Once established the recirculation flow and the length of aerated and non-aerated period, the study of effect of the hydraulic retention time (HRT) on the removal efficiencies of Total-\'N\' and COD was started. The best operating condition was achieved with HDT of 12 hours, with removal efficiency of Total-\'N\' and COD removal of 82% of 89% respectively. Under such condition, the system exhibited high stability with little variation of the monitoring parameters. On the other hand, when the system was operated with HDT of 8 hours and 10 hours, there was a decrease in removal efficiencies of Total-\'N\', to 49% and 45% respectively. Regarding COD removal, the efficiencies at HDT of 8 hours and 10 hours were 85% and 88% respectively, indicating that organic matter removal efficiency is not limited in such range of HDT. The low concentration of suspended solids in the effluent indicates the biomass was firmly attached to the bed. Kinetic assays were performed to determine the rates of nitrification and denitrification in the reactor operating at its best operational condition. Temporal profiles obtained from batch reactors allowed for obtaining an ammonia oxidation rate of 1.43 mg\'N\'/gVSS.h. The nitrite oxidation rates were 1.87 mg\'N\'/gVSS.h and 1.3 mg\'N\'/gVSS.h. The reduction rates of nitrate to nitrite were 3.4 mg\'N\'/gVSS.h and 4.0 mg\'N\'/gSSV.h. The denitrification rate of nitrite to \'N IND.2\' were 2.3 mg\'N\'/gSSV.h and 2.9 mg\'N\'/gSSV.h.

Application of multisource feedback (MSF) increased dramatically and became widespread globally in the past two decades, but there was little conceptual work regarding self-other agreement and few empirical studies investigated self-other agreement in other cultural settings. This study developed a new conceptual framework of self-other agreement and used three samples to illustrate how national culture affected self-other agreement. These three samples included 428 participants from China, 818 participants from the US, and 871 participants from globally dispersed teams (GDTs). An EQS procedure and a polynomial regression procedure were used to examine whether the covariance matrices were equal across samples and whether the relationships between self-other agreement and performance would be different across cultures, respectively. The results indicated MSF could be applied to China and GDTs, but the pattern of relationships between self-other agreement and performance was different across samples, suggesting that the results found in the U.S. sample were the exception rather than rule. Demographics also affected self-other agreement disparately across perspectives and cultures, indicating self-concept was susceptible to cultural influences. The proposed framework only received partial support but showed great promise to guide future studies. This study contributed to the literature by: (a) developing a new framework of self-other agreement that could be used to study various contextual factors; (b) examining the relationship between self-other agreement and performance in three vastly different samples; (c) providing some important insights about consensus between raters and self-other agreement; (d) offering some practical guidelines regarding how to apply MSF to other cultures more effectively.

A Estenose de Canal Lombar (ECL) é caracterizada pela redução do espaço de elementos neurais ou vasculares da coluna lombar. Os sintomas incluem dor e/ou parestesia em membros inferiores, região lombar, e glúteos e Claudicação Intermitente Neurogênica. O tratamento conservador é a escolha inicial para o manejo de pacientes com ECL, por isso é importante a identificação de diferentes estratégias para o tratamento conservador, e a compreensão das alterações em parâmetros funcionais que estes tratamentos geram. O presente estudo objetivou comparar os efeitos de 24 sessões de Fisioterapia Aquática (GFA) e de Fisioterapia Solo (GFS) nos parâmetros espaço-temporais de marcha, equilíbrio, nível de dor percebida, incapacidade, capacidade física funcional, medo de movimento e níveis de depressão em indivíduos com ECL. Para observação das diferenças entre os grupos e momentos foi realizado o teste de Equações de Estimativa Generalizada no software SPSS versão 20.0. Para todas as análises o índice de significância adotado foi de 95% (α=0,05). Os níveis de incapacidade, dor, e estados depressivos reduziram, bem como a velocidade de caminhada autosselecionada e o índice de reabilitação locomotora aumentaram após as intervenções sem diferenças entre os grupos. O GFA apresentou melhora dos parâmetros espaço-temporais de marcha em velocidade fixa, redução da ingesta de medicação analgésica, e redução da média semanal de dor mais cedo que o GFS. O GFS demonstrou maior melhora da mobilidade funcional indicando um melhor equilíbrio após a intervenção em comparação ao GFA. Tratamentos fisioterapêuticos estruturados com associação de exercícios de estabilização de tronco, alongamentos e terapia manual mostram-se eficazes para melhora da incapacidade, dor, depressão e parâmetros de marcha em indivíduos com Estenose da Canal Lombar, sendo o meio aquático potencializador da resposta analgésica e da melhora em parâmetros espaço temporais de marcha e o solo potencializador da melhora do equilíbrio. / Lumbar Spinal Stenosis (LSS) is characterized by a reduction in the space of vascular and neuronal elements of the spinal canal space. Symptoms include pain and/or paresthesia in lower limbs, lumbar region and buttocks, and Neurogenic Intermittent Claudication. Conservative treatments are the choice for initial management of patients with LSS, therefore it is important to identify different conservative treatments strategies and understanding of the changes in functional parameters that those treatments cause. The present study aimed to compare the effects of 24 physiotherapy sections of Aquatic Physiotherapy (GFA) and Ground Physiotherapy (GFS) in spatiotemporal gait parameters, balance, perceived level of pain, disability, functional exercise capacity, fear of movement, and clinical depression in individuals with LSS. Generalized Estimating Equations were made to observe the differences between groups and moments on SPSS software version 20.0. For all tests, the significance level was 95% (α = 0,05). Disability, pain and depressive symptoms reduced and selfselected walking speed and locomotor rehabilitation index increased after interventions with no differences between groups. GFA improve spatiotemporal gait parameter in fixed speed, reduced analgesic medication, and show an earlier reduction in weekly pain. GFS improve functional mobility indicating a better balance in comparison to GFA. Physiotherapeutic treatments structured with the association of stabilization exercises, stretching exercises and manual therapy are efficient in improving disability, pain, depression and gait parameters in Lumbar Spinal Stenosis patients, water environment potentiate analgesic and spatiotemporal improvements, and land potentiate balance improvements.

La porphyrie aiguë intermittente (PAI) est la plus fréquente des porphyries hépatiques aiguës. Elle est décrite comme une maladie autosomique dominante dont le trait génétique est estimé à 1/1675 en France avec une pénétrance faible et variable allant de 10% à 50% dans les familles connues de PAI. La PAI est due à des mutations réduisant le niveau d’activité de l’hydroxyméthylbilane-synthase (HMBS). Son déficit entraîne l’accumulation de précurseurs neurotoxiques responsables de la symptomatologie clinique. Dans le foie, la synthèse d’hème est contrôlée par l’enzyme ALA-Synthétase 1 (ALAS1) dont l’activité est régulée par un rétrocontrôle négatif par le produit final : l’hème. Le traitement consiste à freiner l’induction d’ALAS1 induit par la carence en hème, par l’administration d’hème exogène. Ce traitement de la crise aiguë est très efficace mais génère rapidement une dépendance physique avec apparition de crises récurrentes nécessitant l’administration chronique d’hème exogène. L’objectif principal de ce projet a été d’étudier les mécanismes physiopathologiques et génétiques liés à cette pathologie afin de traiter et conseiller au mieux les patients. Une partie du projet a consisté à explorer les facteurs génétiques modulateurs de la pénétrance de la maladie. Tout d’abord, une prévalence minimale du trait génétique dans la population générale a été estimée à 1/1299 permettant d’en déduire une pénétrance de l’ordre de 1% alors que celle dans les familles PAI suivies par le CFP est estimée à 22,9 %. Ensuite, concernant les facteurs pouvant expliquer cette différence, la présence d’une mutation type non-sens est plus fréquemment associée aux formes sévères et à une pénétrance plus élevée. De plus, les études de corrélation et d’héritabilité suggèrent plutôt une transmission de type oligogénique associée à des facteurs épigénétiques modulateurs de la pénétrance dont le facteur environnemental. Une autre partie a consisté à explorer les effets de l’administration d’hème exogène sur les patients et un modèle murin de PAI créé génétiquement. Chez l’homme, le traitement est associé à une augmentation des formes chroniques (1,7 % avant vs 7,5 % après l’introduction du celui-ci). Dans le modèle murin de PAI, les injections intrapéritonéales répétées induisent une augmentation paradoxale d’ALAS1 (3 fois), une augmentation de l’hème oxygénase 1 qui catabolise l’hème (HMOX1, 9 fois) ainsi que des voies de l’inflammation (analyse transcriptomique et protéomique hépatique) et une surcharge en fer. De plus, cette administration induit une altération des complexes de la chaine respiratoire mitochondriale responsable d’anomalies du métabolisme énergétique au niveau hépatique, cérébral et musculaire pouvant expliquer la symptomatologie neuroviscérale. En conclusion, ce travail a permis d’explorer les caractéristiques génétiques de la maladie (prévalence, pénétrance) en remettant en cause le mode de transmission autosomique dominant jusqu’ici admis, et d’explorer les mécanismes physiopathologiques associées à l’administration d’hème exogène faisant de cette thérapeutique un pharmakon / The biosynthesis of porphyrins is one of the most conserved pathways known. By associating different metals, porphyrins give rise to the "pigments of life". The formation of haem is accomplished by a sequence of eight dedicated enzymes encoded by different genes, some being active in ubiquitous as well as in erythroid isoforms. In humans, the genes for each of the haem synthetic enzymes may become the target of mutations that give rise to an impaired cellular enzyme activity called porphyrias. The acute porphyrias are characterized by attacks of neuropsychiatric symptoms, which may be due to a toxic surplus of the porphyrin precursor 5-aminolevulinic acid, or a consequence of a deficit of vital hemoproteins. Mutations of the gene encoding the third enzyme: hydroxymethylbilane synthase, are associated with the most frequent type of acute hepatic porphyria, acute intermittent porphyria. AIP is thought to display autosomal dominant inheritance with incomplete penetrance. In the classical form of AIP, HMBS activity is about 50% lower than normal in all tissues. These levels of activity in basal conditions are not sufficiently low to cause symptoms. However, factors increasing hepatic heme demand, resulting in an upregulation of hepatic aminolevulinate synthase (ALAS1, the first enzyme of the heme biosynthesis pathway), precipitate acute attacks. The treatment of the attack of AIP consists to repress ALAS1 and restores metabolic equilibrium. But this treatment leads side effects and dependency. The pathophysiological mechanism of the disease is partially known and difficult to explore because there is not an AIP model or prediction model of porphyrogenicity. We aimed to obtain further insight into the pathophysiological mechanism of AIP and into the genetic (prevalence and penetrance) of AIP, and the contribution of genetic factors to the variable clinical expression of HMBS mutations.We first calculated the penetrance of HMBS mutations in AIP patients seen at the French reference center for porphyria: 22.9%. We then used the Exome Variant Server (EVS) to estimate the prevalence of deleterious HMBS mutations in the general population: 1/1299; and the penetrance of the AIP genetic trait in France: 1%. Finally, we investigated further the genetic factors underlying the penetrance of AIP by analyzing genotype/phenotype correlations, and the pattern of familial correlations for the symptoms of the acute crises of AIP. Intrafamily correlation studies showed correlations to be strong overall and modulated by kinship and the era in which the person was living, demonstrating strong influences of genetic and environmental modifiers on inheritance suggesting that AIP inheritance does not follow the classical autosomal dominant model. Null alleles were associated with a more severe phenotype and a higher penetrance than for other mutant alleles.On the other hand, we explored the effect of heme administration. In human, the introduction of hemin into the pharmacopeia has coincided with a 4.4-fold increase in the prevalence of chronic patients. We show that repeated hemin infusions in mice trigger a high level heme oxygenase 1 response, induce a pro-oxidative iron accumulation, a complex pattern of liver inflammation with macrophage infiltration and an alteration of oxidative phosphorylation

L'informatique s'est récemment développée autour de deux axes : l'informatique ambiante d'une part avec la multiplication des objets communicants, et l'internet des services d'autre part suite à l'essor parallèle des centres de traitement de données et d'Internet. Dans ces domaines, la disponibilité fluctuante des ressources, qui entraîne une intermittence des services fournis, représente désormais une préoccupation majeure dans la conception d'applications. La programmation orientée composants appliquée aux architectures orientées service simplifie la gestion des liaisons de service via des politiques. Néanmoins les politiques de liaison existantes suivent soit une approche statique interdisant alors toute reconfiguration d'architecture en cours d'exécution, soit une approche dynamique ne garantissant pas une stabilité minimale de l'architecture dans le cas de services intermittents. Cette thèse propose un compromis entre stabilité architecturale et dynamisme en plaçant l'interruption de service au centre des préoccupations du concepteur. La politique de liaison résultante offre ainsi une tolérance aux interruptions de service jusqu'à une certaine limite au-delà de laquelle l'architecture est reconfigurée dynamiquement. Afin de situer cette limite, notre proposition se base sur l'utilisation d'accords de niveau de service, une forme enrichie des contrats de service permettant, entre autres, l'expression de contraintes sur la disponibilité des services. L'approche a été expérimentée sur la plate-forme à services OSGi en étendant les mécanismes de gestion des liaisons du modèle à composants iPOJO, puis validée dans le contexte de l'informatique ambiante ainsi que sur le serveur d'applications JOnAS.

Energy storage technologies have the potential to revolutionize the electric grid by allowing for the integration of renewable generation while increasing the utilization and efficiency of current grid assets. These technologies include pumped hydroelectric storage, compressed air energy storage (CAES), flywheels, batteries, thermal energy storage (TES), super capacitors, and superconducting magnetic energy storage (SMES). While energy storage has been implemented in some areas, its benefits are greatly undervalued by current regulatory frameworks leading to suboptimal outcomes for grid operators, utilities, and ratepayers. Large-scale adoption of storage technologies will require regulatory frameworks that recognize the benefits of grid-scale storage across generation, and transmission and distribution. This thesis discusses the need for storage, currently available and developing storage technologies, and the present regulatory environment.

Two-phase flow, boiling, and condensation in microchannels have received considerable attention in the recent past due to the growing interest in the high heat fluxes made possible by these channels. This dissertation presents a study on the condensation of refrigerant R134a in small hydraulic diameter (100 < Dh < 160 mm) channels. A novel technique is used for the measurement of local condensation heat transfer coefficients in small quality increments, which has typically been found to be difficult due to the low heat transfer rates at the small flow rates in these microchannels. This method is used to accurately determine pressure drop and heat transfer coefficients for mass fluxes between 300 and 800 kg/m2-s and quality 0 < x < 1 at four different saturation temperatures between 30 and 60oC. The results obtained from this study capture the effect of variations in mass flux, quality, saturation temperature, hydraulic diameter, and channel aspect ratio on the observed pressure drop and heat transfer coefficients. Based on the available flow regime maps, it was assumed that either the intermittent or annular flow regimes prevail in these channels for the flow conditions under consideration. Internally consistent pressure drop and heat transfer models are proposed taking into account the effect of mass flux, quality, saturation temperature, hydraulic diameter, and channel aspect ratio. The proposed models predict 95% and 94% of the pressure drop and heat transfer data within ±25%, respectively. Both pressure drop and heat transfer coefficient increase with a decrease in hydraulic diameter, increase in channel aspect ratio and decrease in saturation temperature. A new non-dimensional parameter termed Annular Flow Factor is also introduced to quantify the predominance of intermittent or annular flow in the channels as the geometric parameters and operating conditions change. This study leads to a comprehensive understanding of condensation in microchannels for use in high-flux heat transfer applications.