Résumé : Introduction : L’ataxie récessive spastique de Charlevoix-Saguenay (ARSCS) est une maladie neuromusculaire héréditaire rare qui affecte notamment les voies spinocérébelleuses. Elle est caractérisée par une triple composante de signes et symptômes incluant l’ataxie et la dysarthrie (atteintes cérébelleuses), la spasticité aux membres inférieurs (atteintes pyramidales) et une faiblesse distale qui engendre des difficultés de préhension (atteintes neuropathiques). Des avancées récentes permettent de croire que des essais thérapeutiques seront bientôt possibles. Dans ce contexte, il est nécessaire de développer une échelle de gravité de la maladie pour permettre la sélection des patients et la documentation de l’histoire naturelle. But : L’objectif de ce projet était de développer les items de la section cérébelleuse du Disease Severity Index for Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (DSI-ARSACS) et de documenter ses qualités métrologiques. Méthode : La section cérébelleuse a été élaborée à l’aide du modèle de développement de Streiner et Norman (2008) qui comprend trois grandes étapes : planification, construction et validation. La planification et la construction ont été réalisées à l’aide de consultations d’experts par la méthode Delphi et d’une recension systématique des écrits. La validité de construit (convergente et discriminatoire) et la fidélité (intra-évaluateur et inter-évaluateur) ont été documentées. Vingt-huit participants ont été recrutés selon un échantillonnage stratifié pour l’âge et le sexe. Ils devaient avoir un diagnostic confirmé génétiquement et être âgés entre 18 ans et 59 ans. La validité convergente a été documentée avec des outils évaluant les aptitudes motrices des membres supérieurs (9HPT, PPT, TDNS), la gravité de l’ataxie (SARA), la mobilité (6MWT, 10mWT, échelle de Berg), le fonctionnement dans les activités quotidiennes (Index de Barthel), la participation sociale (MHAVIE) et la qualité de vie (SF-12v2). La validité discriminante a été documentée selon le sexe, le groupe d’âge et le stade de la maladie. La section cérébelleuse a été appliquée à trois reprises par deux physiothérapeutes à deux semaines d’intervalle pour évaluer la fidélité intra et inter-évaluateurs. Résultats : La section cérébelleuse comporte 6 items d’évaluation liés aux fonctions motrices du cervelet. Le sous total de la section cérébelleuse est fortement corrélé avec la majorité des outils d’évaluation (r ≥ 0,69, p = 0,00), à l’exception du SF-12v2 (r ≤ 0,36, p ≥ 0,06). Une différence significative (p < 0,00) a été démontrée entre chaque groupe d’âge pour la majorité des items et le sous-total de la section cérébelleuse de l’échelle. Le résultat de chaque item et le sous-total de la section cérébelleuse augmentent significativement avec le stade de la maladie (p < 0,00). La fidélité intra et inter-évaluateurs correspond à un accord fort (κ ≥ 0,69) pour la majorité des items et le sous-total. Conclusion : Ce projet a permis de développer la section cérébelleuse de l’échelle de gravité DSI-ARSACS avec de bonnes qualités psychométriques (validité et fidélité). L’échelle pourra être utilisée dans le processus de catégorisation et d’évaluation des participants pour de futurs essais thérapeutiques. / Abstract : Introduction: Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS) is a rare hereditary neuromuscular disease that mainly affects the spinocerebellar tract. It is characterized by a triad of signs and symptoms, including ataxia and dysarthria (cerebellum impairment), spasticity in the lower limbs (pyramidal impairment) and a distal weakness leading to prehension difficulties (neuropathic impairment). Recent research advances suggest that clinical trials may soon become possible. In this context, it is necessary to develop a disease severity index in order to select patients and document natural history of disease. Objective: The project aimed to develop the items of the cerebellar section of the Disease Severity Index for Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay DSI-ARSACS and document their metrological properties. Method: The cerebellar section was developed using the Streiner and Norman (2008) model, which involves three stages: planning, construction and validation. The planning and construction stages were conducted by carrying out a literature review, obtaining expert opinions and completing a Delphi process. Construct validity (convergent and discriminant) and reliability (intra- and inter-raters) were documented. Twenty-eight participants between the ages of 18 and 59 were recruited using a stratified sampling method based on age and gender. All of them had a genetically confirmed diagnosis of ARSACS. Convergent validity was documented with measurements of upper limbs motor abilities (9HPT, PPT, TDNS), ataxia severity (SARA), mobility (6MWT, 10mWT, Berg scale), functional status in activities of daily living (Barthel index), social participation (MHAVIE) and quality of life (SF-12v2). Discriminant validity was documented according to gender, age group and disease stage. The cerebellar section was administered three times, two weeks apart, by two physiotherapists to assess intra- and inter-rater reliability. Results: The cerebellar section includes 6 assessment items linked to cerebellum motor functions. The cerebellar section subscore was strongly correlated with the majority of assessment tools (r ≥ 0.69, p = 0.00), excluding SF 12v2 (r ≤ 0,36, p ≥ 0,06). A significant difference (p < 0.00) was observed between each age group for most items and the subscore of the cerebellar section. Results on each item and the subscore significantly increase with disease stage (p < 0.00). Intra- and inter-rater reliability reflects a strong level of agreement (κ ≥ 0.69) on the majority of items and the subscore. Conclusion: The cerebellar section of the DSI-ARSACS shows good psychometric properties (validity and reliability). The index can be used to categorize and assess future participants in clinical trials.

由近期的研究中發現變動所有參數的管制圖在偵測小幅度偏移時的速度比起傳統的舒華特管制圖來的快，許多文獻也討論到利用調適性管制技術同時監控製程的平均數和變異數。而在這份研究中，為了改善現有管制圖的偵測效率，依序提出了U-V管制圖以及Max-M管制圖來偵測單一製程與兩相依製程的平均數和變異數。採用AATS及ANOS來衡量管制圖的偵測績效，並利用馬可夫鏈推導計算得之。透過兩階段的範例來介紹所提出的管制圖的應用方法並將VP U-V管制圖、VP Max-M管制圖與FP Z(X-bar)-Z(Sx^2)管制圖加以比較。從所研究的數值分析中發現VP Max-M管制圖比另兩種管制圖的表現來的好，再加上只需要單一管制圖在使用上對工程師來說也較為簡便，因此建議Max-M管制圖値得在實務上被使用。 / Recent studies have shown that the variable parameters (VP) charts detect small process shifts faster than the traditional Shewhart charts. There have been many papers discussed adaptive control schemes to monitor process mean and variance simultaneously. In the study, to improve the efficiency and performance of the existing control charts, the U-V control charts and Max-M control charts are respectively proposed to monitor the process mean and variance for a single process and two dependent process steps. The performance of the proposed control charts is measured by using adjusted average time to signal (AATS) and average number of observations to signal (ANOS). The calculation of AATS and ANOS is derived by Markov chain approach. The application of the proposed control charts is illustrated by a numerical example for two dependent process steps, and the performance of VP U-V control charts, VP Max-M control charts and FP Z(X-bar)-Z(Sx^2) control charts is compared. From the results of data analyses, it shows that the VP Max-M control charts have better performance than VP U-V control charts and FP Z(X-bar)-Z(Sx^2) control charts. Furthermore, using a single chart to monitor a process is easier than using two charts for engineers. Hence, Max-M control charts are recommended in real industrial process.

Les interactions protéine-protéine jouent un rôle primordial dans de nombreux processus biologiques. L’importance de ces interactions a suscité le développement de nouvelles approches thérapeutiques qui ciblent ces complexes protéiques. Nous nous proposons d’inhiber ces interactions en élaborant une stratégie de reconnaissance de surfaces de protéines par des molécules synthétiques de taille intermédiaire, les foldamères d’oligoquinoline. Ces composés se replient en des structures hélicoïdales stables dont chaque élément constitutif peut être fonctionnalisé pour permettre des propriétés de reconnaissance de surface de protéine.Afin de valider ce concept, l’interaction entre l’anhydrase carbonique humaine de type II (HCAII) et son inhibiteur N-benzyl-4-sulphamoylbenzamide (SBB) a été sélectionnée comme système modèle. Plusieurs étapes de synthèse ont permis de concevoir de nouveaux foldamères capables de former un complexe avec l’enzyme par l’intermédiaire de l’inhibiteur SBB et d’un espaceur approprié. Chaque complexe protéine-foldamère a été co-cristallisé et l’affinité des interactions a été caractérisée par dichroïsme circulaire induit et par résonance plasmonique de surface. Ce concept a ensuite été appliqué à une interaction protéine-protéine d’intérêt thérapeutique, le complexe IL-4/IL-4R, dans le cadre du programme européenFOLDAPPI (FP7-PEOPLE-IAPP-2008). / Protein-protein interactions play key roles in many biological processes as well as in many diseases. The importance of these interactions has led to the development of new therapeutic approaches that target protein interfaces. We have developed a protein surface recognition strategy to inhibit protein-protein interactions by using intermediate size organicmolecules called oligoquino line foldamers, that result in very stable and well defined helical structures. These helical backbones are used as templates within each building block can be modulated to allow protein surface recognition.In order to validate this concept, the well-characterized interaction between the enzyme human carbonic anhydrase II (HCAII) and its N-benzyl-4-sulphamoylbenzamide (SBB) inhibitor was selected as a model system. Multi-steps synthesis allowed functionalization of new foldamers able to bind to the enzyme through the SBB inhibitor attached by a spacer.Each foldamer–protein complex was cocrystallized and the affinity of the interactions was assayed using both induced circular dichroïsm and surface plasmon resonance. The concept of using a foldamer against protein-protein interaction was then applied to a protein complex of therapeutic interest, IL-4/IL-4R, within the European FOLDAPPI program (FP7-PEOPLEIAPP- 2008).

Title of a document: Floody analysis of Czech republic between 1997 - 2007. Aim of a document: Characterize kinds of extraordinary events and assign to them some cases from CR with concentraining on floods. The legislature before and after acceptance of crisis laws. The economic incidence and loses of lives in effect of floods. Method: An analysis of obtained information oriented to a question of extraordinary events with concentraining on floods. Interview with the specialist of joint rescue service. Results: Complexed summary of extraordinary events, their characterizations and cases reffered to CR with concentraining on floods. Floody analysis of CR between 1997 - 2007 from aspekt of rise, progress and their results. Key words: Joint rescue service, an extraordinary event, crisis situation, rescue and liquidation works, flood, crisis kontrol, economic steps, crisis laws.

Made available in DSpace on 2016-04-25T20:21:04Z (GMT). No. of bitstreams: 1 Egydio Barbosa Zanotta.pdf: 1601956 bytes, checksum: 19d6138c932f32f13bd5155a613801bb (MD5) Previous issue date: 2013-10-25 / The main objectiv of this study is to present a contribution to the non sampling error in the Marketing Research. To reach this goal we had ( by means of bibliografical research) to discover and typify the non sampling error which is present in a research project, assuming the presence of this error in each step of the process. Finally we tried to solve these problems by taking into account the more recents conquests of the knowledge in this area. It is important to emphasize that we included notes and theories originated from our own experience, stimulated by the opinion of the advisor of our thesis / O objetivo central desse estudo é de proporcionar Uma Contribuição Para o Estudo Sobre o Erro Não Amostral na Pesquisa de Mercado. Para lograr atingir esse objetivo, necessitamos através da pesquisa bibliográfica, descobrir e tipificar os erros não amostrais existentes no projeto de pesquisa e, partindo do pressuposto de que em cada etapa, tais erros estão presentes.Posteriormente, passamos a pesquisar como solucionar esses erros, com base no estado da arte. Convém salientar, que graças ao incentivo recebido de minha orientadora, incluímos notas sobre teorias oriundas de nossa experiência e conhecimento

Made available in DSpace on 2014-12-17T15:39:06Z (GMT). No. of bitstreams: 1 AdrianaCFL_DISSERT.pdf: 1171227 bytes, checksum: adc6aafe2083e0cbd20d3b65fa7adff9 (MD5) Previous issue date: 2014-02-21 / Psychological assessment is a procedure that is inserted in all areas of a psychologist s professional field of work. When starting any psychological intervention, it is necessary to carry an analysis of how individual operate in order to identify and define their demands to adequately meet them. It is important to study this practice because the choice of instruments, techniques and procedures to be employed influences the efficiency and effectiveness of the work and the image of the profession towards society in general, also impacting the scientific community. This study aimed to identify, in the subjects, the percentage of professionals in psychology who make use of the Psychological Assessment in the Northeastern region of Brazil; another objectives were to analyze the profile of these professionals in terms of gender, time after graduation, additional training in PA, the state of the country where he/she works; to check how psychologists do their planning when using PA as well as to identify which differences can be observed in terms of time from graduation and additional training in the process of planning the assessment different extents. The instrument used was an online questionnaire and the sample was by adhesion to the application. The attendance was of 159 professionals and most of them were females with a specialist degree. It was found that the majority of the professionals perform the planning of the PA, establishing a method and choosing the most appropriate strategies to be used. It was also verified that most of the subjects fulfill the steps of the whole process, and when planning the techniques of data collection, integrating database from different sources, reporting the results and returning the information, the professional demonstrates his/her goals to understand their demands and accomplish the decision making and/or proposed intervention for individuals assessed. It was found that the undergraduate degree may not have produced a more complete effect in the professional qualification and therefore, of the quality of the actions in PA, and that there is a need for training and continuing education / A avalia??o psicol?gica ? um procedimento que est? inserido em todas as ?reas de atua??o profissional do psic?logo. Ao se iniciar qualquer interven??o psicol?gica, faz-se uma an?lise do funcionamento do(s) indiv?duo(s) visando identificar e definir suas demandas para atender adequadamente. Importa estudar essa pr?tica, pois a decis?o de instrumentos, procedimentos e t?cnicas a empregar influencia a efici?ncia e efic?cia dos trabalhos bem como a imagem da profiss?o na sociedade em geral, repercutindo tamb?m na comunidade cient?fica. Objetivou-se identificar nos respondentes, o percentual de profissionais em Psicologia que fazem uso da Avalia??o Psicol?gica na Regi?o Nordeste do pa?s; analisar o perfil desses profissionais em termos de g?nero, quanto tempo tem de graduado, forma??o adicional em AP, estado onde atua; verificar como, os psic?logos realizam seu planejamento ao atuarem num processo de AP e identificar quais diferen?as podem ser verificadas em termos de tempo de atividade, forma??o complementar no planejamento do processo avaliativo em distintos ?mbitos. O instrumento utilizado foi um question?rio online e a amostra foi por ades?o a proposta. Participaram 159 profissionais sendo a maioria do sexo feminino, com t?tulo de especialista. Identificou-se que a maioria dos profissionais realiza o planejamento da AP, estabelecendo um m?todo e escolhendo as estrat?gias mais adequadas a serem utilizadas. Tamb?m identificou-se que, a maioria cumpre as etapas desse processo, e, ao planejar as t?cnicas de coleta de informa??es, integrar os dados provenientes de diferentes fontes, relatar resultados e executar a devolu??o de informa??es, o profissional demonstra seu objetivo em compreender sua demanda e realiza a tomada de decis?o e/ou proposta de interven??o em rela??o ?s pessoas avaliadas. Verificou-se que a gradua??o possa n?o ter um efeito mais pronunciado na qualifica??o profissional e consequentemente na qualidade das a??es na AP, mas, a necessidade de uma qualifica??o e forma??o continuada

Made available in DSpace on 2016-04-26T14:54:35Z (GMT). No. of bitstreams: 1 Egydio Barbosa Zanotta.pdf: 1601956 bytes, checksum: 19d6138c932f32f13bd5155a613801bb (MD5) Previous issue date: 2013-10-25 / The main objectiv of this study is to present a contribution to the non sampling error in the Marketing Research. To reach this goal we had ( by means of bibliografical research) to discover and typify the non sampling error which is present in a research project, assuming the presence of this error in each step of the process. Finally we tried to solve these problems by taking into account the more recents conquests of the knowledge in this area. It is important to emphasize that we included notes and theories originated from our own experience, stimulated by the opinion of the advisor of our thesis / O objetivo central desse estudo é de proporcionar Uma Contribuição Para o Estudo Sobre o Erro Não Amostral na Pesquisa de Mercado. Para lograr atingir esse objetivo, necessitamos através da pesquisa bibliográfica, descobrir e tipificar os erros não amostrais existentes no projeto de pesquisa e, partindo do pressuposto de que em cada etapa, tais erros estão presentes.Posteriormente, passamos a pesquisar como solucionar esses erros, com base no estado da arte. Convém salientar, que graças ao incentivo recebido de minha orientadora, incluímos notas sobre teorias oriundas de nossa experiência e conhecimento

No description available.

This project asserts that much of the cultural anxiety found in Gothic-infused late-Victorian fiction derives from literary revelations of the nested spaces, shifting identities, and spontaneous connections inherent to the late-Victorian metropolis. The three literary texts studied here – The Hound of the Baskervilles by Arthur Conan Doyle, Raffles: The Amateur Cracksman by E.W. Hornung, and The Thirty-Nine Steps by John Buchan – all depict London as fundamentally suitable for those who seek to evade the disciplinary gaze and to pursue menacing schemes of criminality and invasion. Doyle’s text illustrates the interconnectedness of the spaces within London as well as the passable threshold between London and the English countryside; both the villain Stapleton and the hero Sherlock Holmes use these connections to attack and defend, respectively, the city and its inhabitants. Hornung’s stories depict the machinations employed by the gentleman-thief Raffles as he alters his identity and his codes of behaviour in order to free himself to pursue criminal ends and thus as he challenges cultural barriers. Buchan’s text, building on the others, explores the dissolution of cultural boundaries and identities incumbent upon the spontaneous connections made between those who attack English culture and those, like Richard Hannay, who defend it. There emerges in these texts a vision of London (and by extension Great Britain) as a swirling vortex of motion, an unknowable labyrinth perpetually threatened by menacing agents from without and within. I have employed Victor Turner’s theories of liminality and communitas to describe how criminal agents, and their equally menacing “good-guy” pursuers, separate themselves from structured society in order to move freely and to gain access to the contested thresholds they seek to infiltrate. I also invoke theories of the Gothic, surveillance, and travel, as well as Jeffrey Cohen’s monster theory, to characterize the anxiety embedded in such invasions. / The transformation of contested space : Baker Street, Grimpen Mire and the battle for thresholds in The hound of the Baskervilles -- Hornung's code-switching monster : threatening ambiguity and liminoid mobility in Raffles, the amateur cracksman -- Towards a more inclusive Britishness : Richard Hannay's transformative connections and evolving identity in The thrity-nine steps. / Department of English

近年來有許多研究發現，適應性管制圖在偵測製程或產品幅度偏移時的速度比傳統的舒華特管制圖來的快，許多文獻也討論到利用適應性管制技術同時監控製程的平均數和變異數。隨著科技的發達，許多產品在製造上更加精密，現今普遍使用的固定參數管制圖並無法有效率的偵測出製程失控，導致巨大的成本損失。為了改善現有管制圖的偵測效率與有效控制製程失控下的損失，我們提出了三種適應性加權損失管制圖，包括變動抽樣間隔(VSI)、變動樣本數與抽樣間隔(VSI)、變動管制參數(VP)來偵測單一製程與兩相依製程的平均數和變異數。採用製程發生變動後到管制圖偵測出異常訊息所需的平均時間(AATS)與所需的總觀測數(ANOS)來衡量管制圖的偵測績效，並利用馬可夫鏈推導計算得之。從數值分析中發現，適應性加權損失管制圖在「偵測小偏移幅度時的偵測效率」與「成本的控制」明顯比傳統管制圖表現的更好，再加上每一個製程僅需採用單一管制圖，對使用者也較為簡便並且容易理解，因此適應性加權損失管制圖在實務上是值得被推薦使用的。 / Recent research has shown that control charts with adaptive features detect process shifts faster than traditional Shewhart charts. In this article, we propose three kinds of adaptive weighted loss (WL) control charts, variable sampling intervals (VSI) WL control charts , variable sample sizes and sampling intervals (VSSI) WL control charts and variable parameters (VP) WL control charts, to monitor the target and variance on a single process step and two dependent process steps simultaneously. These adaptive WL control charts may effectively distinguish which process step is out-of-control. We use the Markov chain approach to calculate the adjusted average time to signal (AATS) and average number of observations to signal (ANOS) in order to measure the performance of the proposed control charts. From the numerical examples and data analyses, we find the adaptive WL control charts have better detection abilities and performance than fixed parameters (FP) WL control charts and FP Z(X-bar)-Z(Sx^2) and Z(e-bar)-Z(Se^2) control charts. We also proposed the optimal adaptive WL control charts using an optimization technique to minimize AATS when users cannot specify the values of the variable parameters. In addition, we discuss the impact of misusing weighted loss of outgoing quality control chart. In conclusion, using a single chart to monitor a process is inherently easier than using two charts. The WL control charts are easy to understand for the users, and have better performance and detection abilities than the other charts, thus, we recommend the use of WL control charts in the real industrial process.