913 resultados para PROGRESSIVE SUPRANUCLEAR PALSY


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Background: The Swiss pig population enjoys a favourable health situation. To further promote this, the Pig Health Service (PHS) conducts a surveillance program in affiliated herds: closed multiplier herds with the highest PHS-health and hygiene status have to be free from swine dysentery and progressive atrophic rhinitis and are clinically examined four times a year, including laboratory testing. Besides, four batches of pigs per year are fattened together with pigs from other herds and checked for typical symptoms (monitored fattening groups (MF)). While costly and laborious, little was known about the effectiveness of the surveillance to detect an infection in a herd. Therefore, the sensitivity of the surveillance for progressive atrophic rhinitis and swine dysentery at herd level was assessed using scenario tree modelling, a method well established at national level. Furthermore, its costs and the time until an infection would be detected were estimated, with the final aim of yielding suggestions how to optimize surveillance. Results: For swine dysentery, the median annual surveillance sensitivity was 96.7 %, mean time to detection 4.4 months, and total annual costs 1022.20 Euro/herd. The median component sensitivity of active sampling was between 62.5 and 77.0 %, that of a MF between 7.2 and 12.7 %. For progressive atrophic rhinitis, the median surveillance sensitivity was 99.4 %, mean time to detection 3.1 months and total annual costs 842.20 Euro. The median component sensitivity of active sampling was 81.7 %, that of a MF between 19.4 and 38.6 %. Conclusions: Results indicate that total sensitivity for both diseases is high, while time to detection could be a risk in herds with frequent pig trade. From all components, active sampling had the highest contribution to the surveillance sensitivity, whereas that of MF was very low. To increase efficiency, active sampling should be intensified (more animals sampled) and MF abandoned. This would significantly improve sensitivity and time to detection at comparable or lower costs. The method of scenario tree modelling proved useful to assess the efficiency of surveillance at herd level. Its versatility allows adjustment to all kinds of surveillance scenarios to optimize sensitivity, time to detection and/or costs.

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INTRODUCTION The transcription factor activating enhancer binding protein 2 epsilon (AP-2ε) was recently shown to be expressed during chondrogenesis as well as in articular chondrocytes of humans and mice. Furthermore, expression of AP-2ε was found to be upregulated in affected cartilage of patients with osteoarthritis (OA). Despite these findings, adult mice deficient for AP-2ε (Tfap2e(-/-)) do not exhibit an obviously abnormal cartilaginous phenotype. We therefore analyzed embryogenesis of Tfap2e(-/-) mice to elucidate potential transient abnormalities that provide information on the influence of AP-2ε on skeletal development. In a second part, we aimed to define potential influences of AP-2ε on articular cartilage function and gene expression, as well as on OA progression, in adult mice. METHODS Murine embryonic development was accessed via in situ hybridization, measurement of skeletal parameters and micromass differentiation of mesenchymal cells. To reveal discrepancies in articular cartilage of adult wild-type (WT) and Tfap2e(-/-) mice, light and electron microscopy, in vitro culture of cartilage explants, and quantification of gene expression via real-time PCR were performed. OA was induced via surgical destabilization of the medial meniscus in both genotypes, and disease progression was monitored on histological and molecular levels. RESULTS Only minor differences between WT and embryos deficient for AP-2ε were observed, suggesting that redundancy mechanisms effectively compensate for the loss of AP-2ε during skeletal development. Surprisingly, though, we found matrix metalloproteinase 13 (Mmp13), a major mediator of cartilage destruction, to be significantly upregulated in articular cartilage of adult Tfap2e(-/-) mice. This finding was further confirmed by increased Mmp13 activity and extracellular matrix degradation in Tfap2e(-/-) cartilage explants. OA progression was significantly enhanced in the Tfap2e(-/-) mice, which provided evidence for in vivo relevance. This finding is most likely attributable to the increased basal Mmp13 expression level in Tfap2e(-/-) articular chondrocytes that results in a significantly higher total Mmp13 expression rate during OA as compared with the WT. CONCLUSIONS We reveal a novel role of AP-2ε in the regulation of gene expression in articular chondrocytes, as well as in OA development, through modulation of Mmp13 expression and activity.

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PURPOSE OF REVIEW Progressive cardiac conduction disorder (PCCD) is an inherited cardiac disease that may present as a primary electrical disease or be associated with structural heart disease. In this brief review, we present recent clinical, genetic, and molecular findings relating to PCCD. RECENT FINDINGS Inherited PCCD in structurally normal hearts has been found to be linked to genetic variants in the ion channel genes SCN5A, SCN1B, SCN10A, TRPM4, and KCNK17, as well as in genes coding for cardiac connexin proteins. In addition, several SCN5A mutations lead to 'cardiac sodium channelopathy overlap syndrome'. Other genes coding for cardiac transcription factors, such as NKX2.5 and TBX5, are involved in the development of the cardiac conduction system and in the morphogenesis of the heart. Mutations in these two genes have been shown to cause cardiac conduction disorders associated with various congenital heart defects. SUMMARY PCCD is a hereditary syndrome, and genetic variants in multiple genes have been described to date. Genetic screening and identification of the causal mutation are crucial for risk stratification and family counselling.

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In this paper, the scales of Raven's Progressive Matrices Test, General Scale and Advanced Scale, Series II, for the student population (third cycle of EGB and Polimodal ) in the city of La Plata are presented. Considerations are made as regards both the increase in scores (Flynn effect) observed in relation to the previous scale (1964) and the different mean scores according to two age groups (13-16 and 17-18 years of age) and education mode. The findings enabled inferences related to the significance of the increase, particularly in the case of the higher scores in the population attending a special kind of educational institution.

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In this paper, the scales of Raven's Progressive Matrices Test, General Scale and Advanced Scale, Series II, for the student population (third cycle of EGB and Polimodal ) in the city of La Plata are presented. Considerations are made as regards both the increase in scores (Flynn effect) observed in relation to the previous scale (1964) and the different mean scores according to two age groups (13-16 and 17-18 years of age) and education mode. The findings enabled inferences related to the significance of the increase, particularly in the case of the higher scores in the population attending a special kind of educational institution.

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In this paper, the scales of Raven's Progressive Matrices Test, General Scale and Advanced Scale, Series II, for the student population (third cycle of EGB and Polimodal ) in the city of La Plata are presented. Considerations are made as regards both the increase in scores (Flynn effect) observed in relation to the previous scale (1964) and the different mean scores according to two age groups (13-16 and 17-18 years of age) and education mode. The findings enabled inferences related to the significance of the increase, particularly in the case of the higher scores in the population attending a special kind of educational institution.

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We construct an empirically informed computational model of fiscal federalism, testing whether horizontal or vertical equalization can solve the fiscal externality problem in an environment in which heterogeneous agents can move and vote. The model expands on the literature by considering the case of progressive local taxation. Although the consequences of progressive taxation under fiscal federalism are well understood, they have not been studied in a context with tax equalization, despite widespread implementation. The model also expands on the literature by comparing the standard median voter model with a realistic alternative voting mechanism. We find that fiscal federalism with progressive taxation naturally leads to segregation as well as inefficient and inequitable public goods provision while the alternative voting mechanism generates more efficient, though less equitable, public goods provision. Equalization policy, under both types of voting, is largely undermined by micro-actors' choices. For this reason, the model also does not find the anticipated effects of vertical equalization discouraging public goods spending among wealthy jurisdictions and horizontal encouraging it among poor jurisdictions. Finally, we identify two optimal scenarios, superior to both complete centralization and complete devolution. These scenarios are not only Pareto optimal, but also conform to a Rawlsian view of justice, offering the best possible outcome for the worst-off. Despite offering the best possible outcomes, both scenarios still entail significant economic segregation and inequitable public goods provision. Under the optimal scenarios agents shift the bulk of revenue collection to the federal government, with few jurisdictions maintaining a small local tax.

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A paralisia cerebral, doença não progressiva, compromete movimentos e postura. A fisioterapia atual volta-se para um tratamento holístico. Brincar proporciona desenvolvimento neuropsicomotor. O presente estudo tem como objetivos investigar a opinião de fisioterapeutas que atuam em neuropediatria sobre a utilização do brinquedo em sua prática clínica e verificar sua possível utilização em intervenções junto a crianças com paralisia cerebral. Utiliza-se inicialmente de questionário de opinião junto a 50 fisioterapeutas das diversas clínicas da Associação de Apoio a Criança com Deficiência, AACD - SP, verificando a utilização de brinquedos face aos diversos objetivos fisioterapeuticos; a seguir, realiza observação de 60 atendimentos, em fisioterapia aquática e de solo, de crianças com paralisia cerebral, identificando a utilização de cada categoria de brinquedo relativo ao objetivo terapêutico. Os dados obtidos no questionário revelaram em ordem decrescente utilização de: brinquedos sensório-motores 57,4%, para ganho de equilíbrio (E); 22,2% para coordenação motora (CM); 18,5% para aquisições posturais (AP) e 2% para relaxamento muscular (RM). Em relação aos jogos de faz-de-conta: 37% (E); 39% (AP) e 24% (CM).Para os jogos de regras: 54% (E); 35% (CM); 11% (AP). Com os jogos de montagem: 52% (CM); 24% (E); 24% (AP). Os dados da observação revelaram que os principais objetivos terapêuticos visados com utilização de brinquedos foram: alongamento, primeiro 10 ; fortalecimento muscular, equilíbrio e treino de marcha de 10 a 40 . Quanto à modalidade de brinquedo observada houve predomínio do faz de conta no início e no fim da sessão e das demais categorias no meio, de forma intercalada. Os dados da observação coincidiram com os do questionário revelando utilização sistemática de brinquedos com objetivos fisioterapeuticos.(AU)

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A paralisia cerebral, doença não progressiva, compromete movimentos e postura. A fisioterapia atual volta-se para um tratamento holístico. Brincar proporciona desenvolvimento neuropsicomotor. O presente estudo tem como objetivos investigar a opinião de fisioterapeutas que atuam em neuropediatria sobre a utilização do brinquedo em sua prática clínica e verificar sua possível utilização em intervenções junto a crianças com paralisia cerebral. Utiliza-se inicialmente de questionário de opinião junto a 50 fisioterapeutas das diversas clínicas da Associação de Apoio a Criança com Deficiência, AACD - SP, verificando a utilização de brinquedos face aos diversos objetivos fisioterapeuticos; a seguir, realiza observação de 60 atendimentos, em fisioterapia aquática e de solo, de crianças com paralisia cerebral, identificando a utilização de cada categoria de brinquedo relativo ao objetivo terapêutico. Os dados obtidos no questionário revelaram em ordem decrescente utilização de: brinquedos sensório-motores 57,4%, para ganho de equilíbrio (E); 22,2% para coordenação motora (CM); 18,5% para aquisições posturais (AP) e 2% para relaxamento muscular (RM). Em relação aos jogos de faz-de-conta: 37% (E); 39% (AP) e 24% (CM).Para os jogos de regras: 54% (E); 35% (CM); 11% (AP). Com os jogos de montagem: 52% (CM); 24% (E); 24% (AP). Os dados da observação revelaram que os principais objetivos terapêuticos visados com utilização de brinquedos foram: alongamento, primeiro 10 ; fortalecimento muscular, equilíbrio e treino de marcha de 10 a 40 . Quanto à modalidade de brinquedo observada houve predomínio do faz de conta no início e no fim da sessão e das demais categorias no meio, de forma intercalada. Os dados da observação coincidiram com os do questionário revelando utilização sistemática de brinquedos com objetivos fisioterapeuticos.(AU)

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A cross-sectional survey was made in 56 exceptionally healthy males, ranging in age from 20 to 84 years. Measurements were made of selected steroidal components and peptidic hormones in blood serum, and cognitive and physical tests were performed. Of those blood serum variables that gave highly significant negative correlations with age (r > −0.6), bioavailable testosterone (BT), dehydroepiandrosterone sulfate (DHEAS), and the ratio of insulin-like growth factor 1 (IGF-1) to growth hormone (GH) showed a stepwise pattern of age-related changes most closely resembling those of the age steps themselves. Of these, BT correlated best with significantly age-correlated cognitive and physical measures. Because DHEAS correlated well with BT and considerably less well than BT with the cognitive and physical measures, it seems likely that BT and/or substances to which BT gives rise in tissues play a more direct role in whatever processes are rate-limiting in the functions measured and that DHEAS relates more indirectly to these functions. The high correlation of IGF-1/GH with age, its relatively low correlation with BT, and the patterns of correlations of IGF-1/GH and BT with significantly age-correlated cognitive and physical measures suggest that the GH–IGF-1 axis and BT play independent roles in affecting these functions. Serial determinations made after oral ingestion of pregnenolone and data from the literature suggest there is interdependence of steroid metabolic systems with those operational in control of interrelations in the GH–IGF-1 axis. Longitudinal concurrent measurements of serum levels of BT, DHEAS, and IGF-1/GH together with detailed studies of their correlations with age-correlated functional measures may be useful in detecting early age-related dysregulations and may be helpful in devising ameliorative approaches.