986 resultados para Brooke, Charlotte, 1740-1793.
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The relationship between population and government in the City of Buenos Aires is analyzed, focusing on the tensions generated by the arrival of new individuals, a local elite with great interests in commerce and in charge of community affairs, and Spanish functionaries that progressively adopted the ideals of the Bourbon Dynasty, despite also being implicated in local logics. It interests us to observe the governors’ perspectives with respect to everyday developments in the city, their preoccupations and interests, and how they would vary the mechanisms to which they resorted in order to organize daily life, in the period defined between 1740 and 1776.
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BACKGROUND: Brooke-Spiegler syndrome (BSS) is probably an underdiagnosed genodermatosis that predisposes for the development of cylindromas, spiradenomas and trichoepitheliomas mainly of the head and neck. Wide phenotypic variability regarding the number and type of lesions can be observed within a family. Mutations of the CYLD gene are identified in the vast majority of cases and play a key role in BSS pathogenesis. MAIN OBSERVATIONS: Two first degree relatives with numerous erythematous telangiectatic nodules of the scalp present for decades, with recurring tendency regardless the multiple previous excisions. Histopathological review of the lesions revealed predominantly "spiradenocylindromas" in the proband and cylindromas in her sister. The suspicion of BSS was confirmed after detection of a new nonsense germline mutation of CYLD (c.1783C>T pGln 595*) in the proband. CONCLUSIONS: BSS diagnosis can be challenging and is based on clinical-pathological correlation, positive familial association and identification of CYLD mutations. CYLD exerts antineoplastic effects by downregulating intracellular NF-κB signalling pathways. The reported mutation affecting the ubiquitin-specific protease domain leads to a truncated and catalytically inactive enzyme. Despite the expanding list of CYLD mutations no firm genotype-phenotype correlation is known so far. Early recognition and treatment of BSS avoid disfiguring changes like "turban tumor".
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BACKGROUND: Brooke-Spiegler syndrome (BSS) is probably an underdiagnosed genodermatosis that predisposes for the development of cylindromas, spiradenomas and trichoepitheliomas mainly of the head and neck. Wide phenotypic variability regarding the number and type of lesions can be observed within a family. Mutations of the CYLD gene are identified in the vast majority of cases and play a key role in BSS pathogenesis. MAIN OBSERVATIONS: Two first degree relatives with numerous erythematous telangiectatic nodules of the scalp present for decades, with recurring tendency regardless the multiple previous excisions. Histopathological review of the lesions revealed predominantly "spiradenocylindromas" in the proband and cylindromas in her sister. The suspicion of BSS was confirmed after detection of a new nonsense germline mutation of CYLD (c.1783C>T pGln 595*) in the proband. CONCLUSIONS: BSS diagnosis can be challenging and is based on clinical-pathological correlation, positive familial association and identification of CYLD mutations. CYLD exerts antineoplastic effects by downregulating intracellular NF-κB signalling pathways. The reported mutation affecting the ubiquitin-specific protease domain leads to a truncated and catalytically inactive enzyme. Despite the expanding list of CYLD mutations no firm genotype-phenotype correlation is known so far. Early recognition and treatment of BSS avoid disfiguring changes like "turban tumor".
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Entre la fin du régime français et l’adoption de l’Acte constitutionnel par le Parlement de Londres en 1791, le rapport que la noblesse canadienne entretient avec le système judiciaire civil de la colonie change de façon majeure. Les Canadiens doivent s’adapter au nouveau système mis en place par l’administration britannique de la colonie. En Nouvelle-France, les nobles présentaient leurs différends juridiques civils devant le Tribunal royal, régi par la Coutume de Paris ; à partir de la Cession (1763), ce sont officiellement les lois britanniques qui s’appliquent jusqu’au retour des lois civiles françaises en 1774. Après quelques adaptations, la Cour des Plaidoyers communs devient la cour de prédilection des Canadiens, et par conséquent, de l’ancienne élite militaire. Le système judiciaire constitue un élément important de l’étude de l’évolution de la colonie, car l’attitude de la caste élitaire face aux tribunaux est un indicateur de sa capacité d’adaptation et de son degré d’implication dans la vie sociale.
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Entre la fin du régime français et l’adoption de l’Acte constitutionnel par le Parlement de Londres en 1791, le rapport que la noblesse canadienne entretient avec le système judiciaire civil de la colonie change de façon majeure. Les Canadiens doivent s’adapter au nouveau système mis en place par l’administration britannique de la colonie. En Nouvelle-France, les nobles présentaient leurs différends juridiques civils devant le Tribunal royal, régi par la Coutume de Paris ; à partir de la Cession (1763), ce sont officiellement les lois britanniques qui s’appliquent jusqu’au retour des lois civiles françaises en 1774. Après quelques adaptations, la Cour des Plaidoyers communs devient la cour de prédilection des Canadiens, et par conséquent, de l’ancienne élite militaire. Le système judiciaire constitue un élément important de l’étude de l’évolution de la colonie, car l’attitude de la caste élitaire face aux tribunaux est un indicateur de sa capacité d’adaptation et de son degré d’implication dans la vie sociale.
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NGL-projekt vid Högskolan Dalarna inom språkavdelningen som handlar om informella internetbaserade lärmiljöer.
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NGL-projekt vid Högskolan Dalarna inom språkavdelningen som handlar om webbaserade digitala prov.
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NGL-projekt vid Högskolan Dalarna inom språkavdelningen som handlar om pedagogiska metoder i nätbaserad språkundervisning.
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This document contains statistics on economic data, demographic data, industry data, occupation and employment data and education data for the Upper South Carolina Region. Also included is a list and directory of higher educational institutions in the region.
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IntroducciónDetrás del bello título y de las referencias explícitamente froidianas de este libro, el lector descubre en él un importante libro de historia regional. De golpe osamos a plantear una afirmación a manera de previsión, ejercicio que por cierto repugna tanto al historiador, pero que en este caso no deja de ser un tanto riesgoso. El trabajo de A. Taracena probablemente será por muchos años como una de esas referencias trascendentales para todos aquellos que decidan en el futuro plantear un estudio de historia regional.