No description available.

No description available.

No description available.

No description available.

Dentre pacientes com imunodeficiências primárias, existem aqueles com defeitos de fagócitos e outros componentes da imunidade inata. A doença granulomatosa crônica (DGC) é uma imunodeficiência primária (IDP) causada por mutações em um dos componentes protéicos, gp91-phox, p22-phox, p47-phox, p67-phox e p40-phox, da nicotinamida adenina dinucleotídeo fosfato (NADPH) dos fagócitos. Pacientes com DGC apresentam maior susceptibilidade a infecções, assim como hiperinflamação e reação adversa à vacinas como à do Bacilo Calmette-Guérin (BCG), como consequência da atividade microbicida defeituosa dos fagócitos. Por outro lado, a susceptibilidade mendeliana a micobactérias (MSMD) é uma condição que predispõe os pacientes a infecções pelo gênero Mycobacterium sp, levando a infecções graves e por vezes à morte. O objetivo deste trabalho foi realizar o diagnóstico clínico e a análise genético-molecular de pacientes brasileiros com DGC e MSMD. A explosão respiratória de granulócitos foi avaliada pelo ensaio de dihidrorodamina (DHR). A dosagem de citocinas do eixo IL-12/IFN-&#947 foi realizada mediante o ensaio de ELISA após estimulo com lisado de micobactérias (LM), proteína purifica (PPD) e BCG. O DNA genômico dos pacientes foi extraído, amplificado e sequenciado pelo método de Sanger e seqüenciamento completo de exoma. Durante o período de 2014-2018, 181 pacientes com histórico clínico sugestivo de DGC e 75 pacientes com diagnóstico sugestivo de MSMD foram encaminhados ao nosso laboratório. Após avaliação clínica e bioquímica dos pacientes, 23 deles foram diagnosticadas com DGC e 16 com MSMD. A análise genético-molecular permitiu identificar mutações em 14 pacientes com DGC, nove deles com DGC ligada ao cromossomo X (DGC-X) e 5 com DGC autossômica recessiva (DGC-AR). Identificamos mutações em 5 pacientes com MSMD, sendo três delas no receptor de IL-12 e duas no receptor da IL-17. / Among patients with primary immunodeficiencies, there are those with defects in phagocytes and other components of the innate immunity. Chronic granulomatous disease (CGD) is a primary immunodeficiency (PID) caused by mutations in one of the protein components, gp91-phox, p22-phox, p47-phox, p67-phox and p40-phox of the Nicotinamide Adenine Dinucleotide Phosphate (NADPH) oxidase of phagocytes. Patients with CDG are susceptible to infections, as well as hyperinflammation and adverse reactions to vaccines such as Bacilo Calmette-Guérin (BCG) as a consequence of defective phagocytes microbicidal activity. On the other hand, Mendelian susceptibility to mycobacterial diseases (MSMD) is a condition that predisposes patients to infections by the genus Mycobacterium sp , leading to serious infections and sometimes death. The main goal of this study was to perform the clinical diagnosis and genetic-molecular analysis of Brazilian patients with CDG and MSMD. The respiratory burst of granulocytes was evaluated by the dihydrorhodamine (DHR) assay. Cytokine dosing of IL-12 / IFN-&#947 axis was performed by the ELISA assay after stimulation with mycobacterium lysate (LM), purified protein (PPD) and BCG. Patients genomic DNA was extracted, amplified and sequenced by the Sanger method and whole exome sequencing. During the period of 2014 to 2018, 181 patients with a clinical history suggestive of CDG and 75 patients with a diagnosis suggestive of MSMD were referred to our laboratory. After clinical and biochemical evaluation, 23 of them were diagnosed with CDG and 16 with MSMD. Genetic-molecular analysis allowed the identification of mutations in 14 patients with CDG, of those 9 had X-linked DGC (X-CGD) and 5 had autosomal recessive CGD (AR-CGD). Mutations were identified in 5 MSMD patients, three in the IL-12 receptor and two in the IL-17 receptor.

No description available.

Stadtbürgertum in Pressburg und Leutschau zwischen sozialer und nationaler Identität in der zweiten Hälfte des 19. Jahrhunderts

Plato thought that dialectic is the method of philosophical inquiry. Yet there is no agreement between scholars regarding Plato\'s view of dialectic. The dominant interpretation, dating back to Robinson\'s Plato\'s Earlier Dialectic, which I call the \"discontinuous\" interpretation (DI), assumes a significant gap between Plato\'s account of dialectic as it is presented in the course of the dialogues. As such, although Plato considered dialectic as the method of philosophical inquiry, the term \'Dialectic\' is just a façon de parler conveying the method he deemed most suitable at different moments. One should note that (DI) entails the following trilemma: Plato\'s dialectic, as the method of philosophical inquiry, must be identified with either Elenchus (E), Hypothesis (H) or Collection & Division (C&D). For example, Irwin (1988:7) argues that one should identify dialectic with (E) while Benson (2015:238) argues that one should identify dialectic with (H). In contrast with (DI), the goal of this dissertation is to defend a \"continuous\" interpretation (CI): Plato introduced a unified view of dialectic as the method of philosophical inquiry in the Phaedrus. My argument supporting (CI) relies on three main premises: (1) The so-called three methods, (E), (H) and (C&D), are three different procedures of one διαλεκτικὴ τέχνη; (2) Plato\'s διαλεκτικὴ τέχνη is both a method of communication and a method of discovering truth; and (3) The Phaedrus (261a-266b) contains Plato\'s unified view of διαλεκτικὴ τέχνη, conceived as an amalgam of (E), (H) and (C&D), and a method of communication and a method of discovering truth. / Platão pensou que a dialética é o método de investigação filosófica. No entanto, não há consenso entre os estudiosos a respeito da sua visão da dialética. A interpretação dominante, que se remonta ao trabalho de Robinson Plato\'s Earlier Dialectic, que eu chamo de interpretação \"descontínua\" (ID), pressupõe que há uma ruptura na visão de Platão sobre a dialética no decorrer dos seus diálogos. Isto significa que ele considerou a dialética como o método de investigação filosófica, mas o termo \'dialética\' é apenas uma façon de parler para se referir ao método que considerou mais adequado em diferentes momentos. Portanto, (ID) implica o seguinte trilema: Devese identificar a visão de Platão sobre a dialética, enquanto o método de investigação filosófica, com o Elenchus (E), com a Hypotesis (H), ou com a Coleção & Divisão (C&D)? Por exemplo, Irwin (1988: 7) afirma que a dialética deve ser identificada com (E), enquanto Benson (2015: 238) afirma que a dialética deve ser identificada com (H). Em contraste com (ID), o objetivo do presente trabalho é defender uma interpretação \"contínua\" (IC): No Fedro Platão introduz uma visão unificada da dialética como método de investigação filosófica. Meu argumento para defender (IC) está baseado em três premissas: (1) os chamados três métodos, (E), (H) e (C&D) são realmente três procedimentos diferentes de uma διαλεκτικὴ τέχνη; (2) a τέχνη διαλεκτικὴ de Platão é o método de comunicação e descoberta da verdade; e (3) o Fedro (261a-266b) contém a visão unificada de Platão sobre a διαλεκτικὴ τέχνη, concebida como uma amálgama de (E), (H) e (C e D), e o método de comunicação e descoberta da verdade.

Au cours du XXème siècle, l'étude de la politique répressive en URSS a été menée sans utilisation des sources statistiques et des données des organismes internationaux. Ces documents étaient tenus strictement secrets et donc inaccessibles. Avec la chute du mur de Berlin en 1989 et l'effondrement de l'URSS en 1991, le monde se trouve complètement modifié. Ce processus engendre la division de certains états en Europe, la création de nouvelles zones de conflit et aussi la proclamation de l'indépendance de certains pays comme l'Azerbaïdjan en octobre 1991. Ces modifications politiques provoquent des mouvements de population. Essentiellement liée à la sécurité, surtout en raison du conflit arrnéno azerbaïdjanais qui a influencé l'économie, les hommes émigrent et s'installent dans les pays de la CEl, particulièrement en Russie et en Ukraine, mais aussi en Allemagne, en Israël, et ailleurs. Aujourd'hui, les raisons de l'émigration du peuple azerbaïdjanais sont différentes. Notre problématique propose d'étudier, de façon historique et sociologique, l'ensemble des courants migratoires azerbaïdjanais vers la France à l'époque contemporaine, c'est-à-dire de la fin du XIXème siècle. au début du XXIème siècle. Cette question est éclairée par l'étude de mouvements migratoires des pays voisins. L'exemple azerbaïdjanais regroupe différents types d'immigration, c'est pourquoi il nous a paru pertinent et judicieux de l'analyser pour comprendre les enjeux actuels des mouvements de population. De plus, l'étude des flux migratoires azerbaïdjanais est intéressant de par ses caractéristiques culturelles, politiques et surtout économiques et permet d'accéder à une meilleure intelligibilité de nos sociétés. / During the twentieth century, the study of political repression in the USSR was conducted without the use of statistical sources and data from international organizations. These documents were kept strictly secret, and therefore inaccessible. With the fall of the Berlin Wall in 1989 and the collapse of the USSR in 1991, the world is in fact completely altered. This process leads to the division of many countries in Europe, which provokes the arousing of new areas of conflict, and also the proclamation of the in dependence of few countries such as Azerbaijan in October 1991. These political changes caused movements of population. Primarily related to security, mainly due to the Armenian-Azerbaijani conflict, which changed the economy in a significant way, population migrate and settle in the CIS countries, more especially in Russia and in Ukraine, or also in Germany, in Israel and elsewhere. Nowadays, the reasons for the emigration of the Azerbaijani people are different. Our research scrutinizes, sociologically and historically, all Azerbaijani migration flows to France at the contemporary times, thal is to say the late nineteenth century to the early twenty-frrst century. This study is enhanced by analyses of migration of neighbouring countries. The example of Azerbaijan gathers different types of immigration that is the reason why we thought it would be relevant and meaningful to analyze the current issues of population movements. In addition, the study of migration of Azerbaijani is thrilling and stimulating because of ils cultural, political and especially economical, facets for a better understanding of our societies

This thesis comparatively analyses dilemmas arising from the unresolved Georgian-Abkhazian and Moldovan-Transnistrian conflicts. The many existing differences in the nature and dynamics of each conflict point to a sui generis situation, yet, many similarities also exist, including Soviet era legacies. The research premise is based on very considerable resourcing being dedicated to the resolution of these conflicts since their inception, yet no significant conflict transformation (Abkhazia has Russian recognition) has occurred, let alone peace. Given the mandate from the OSCE, EU and UN, the purpose of the thesis is to analyse their involvement. While evidence – local vested interests and strategic interests – illustrates the obvious obstructions, the thesis focuses on the IOs and asks why no tangible successes were achieved. This is done by critically analysing their performance, by assuming that success is often overlooked and by identifying a range of misperceptions. Therefore, by considering conflict transformation and engaging in analysis of many sources, including documents and about five-dozen interviews, the thesis reappraises the ethno-national origins, the geopolitical dimension and the entrenched conflicting party positions in the context of pan-European actors' responses. The analyses of the comparative peace processes illuminate the IOs' own challenges in reaching consensus as well as on the conflicting parties' competing narratives. Answering the question of what to produce first – a political solution to improve societal conditions, or the pursuing exercise of confidence-building measures (CBMs) to settle the status question – is part of the objective. I argue that a typology of success and failure more comprehensively explains IO performance between 1992 and the EU's Vilnius Summit in 2013.