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91.
The aim of this investigation was to identify and characterise pathogenic mutations in a sudden cardiac death (SCD) cohort suspected of cardiomyopathy in persons aged 0-40 years. The study material for the genetic screening of cardiomyopathies consisted of 41 cases and was selected from the case database at the Institute of Forensic Medicine. Mutational screening by DNA sequencing was performed to detect mutations in DNA samples from deceased persons suspected of suffering from hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and arrhythmogenic right ventricle cardiomyopathy (ARVC). A total of 9 of the examined 41 cases had a rare sequence variant in the MYBPC3, MYH7, LMNA, PKP2 or TMEM43 genes, of which 4 cases (9.8%) were presumed to be pathogenic mutations. The presumed pathogenic mutations were distributed with one case of suspected HCM and DCM (MYH7; p.R442H), one case of suspected DCM (LMNA; p.R471H), and two cases of suspected ARVC (PKP2; p.R79X and LMNA; p.R644C). The presented data adds important information on the genetic elements of SCD in the young, and calls for expert pathological evaluation and molecular autopsy in the post-mortem examination of SCD victims with structural anomalies of the heart.  相似文献   
92.
Netherlands International Law Review -  相似文献   
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94.
As part of a special issue on the geographies of citizenship, this paper considers the longstanding and popular metaphor of “the body politic” for a polity. The metaphor's comparative power is successful because it imports key geographic assumptions about how polities are best organized. It makes claims about society and space (premises about location, spatial organization), nature–society relations (how the two spheres do and do not connect) and cartographic representation (the human body is the optimal representation of spatial and natural relations in a polity). We describe three ways in which geographical imaginations are constructed: organic, mechanical, and posthuman bodies politic. Our goal is twofold: first is to consider the ways the deployment of the metaphor of the body is used in political theory to convey a normative conception of citizenship; second is to bridge the gap between political theory and geography by paying special attention to the ways the body is a space. The metaphor of the body politic is a political geography that links citizenship to particular geographical and normative relationships.  相似文献   
95.
Students of legislative behavior are divided over the extent to which an electoral connection existed in the early United States. In this article, we offer a test of the electoral connection in early American politics by investigating the electoral aftershocks of the disputed presidential election of 1824. Using newly available county-level presidential voting data, along with the unique circumstances associated with the presidential contest, we examine the connection between representative behavior, district public opinion, and electoral outcomes. We find that representatives who voted for John Quincy Adams in the House contest, yet were from districts supporting Andrew Jackson, were targeted for ouster and suffered a substantial vote-loss in the subsequent midterm election. We also find that the entry of a quality challenger had a sizeable impact on the fortunes of incumbent legislators. These results serve to confirm that representatives could be held accountable for their behavior in office during the antebellum era .  相似文献   
96.
Many studies of the relationship between the state and interest organizations conclude that the state is captured by sectoral interests. The state is not autonomous in a capitalist society. However, does the neo-corporatist practice necessarily imply a captured state? The author examines the dependencies of the state on functional groups and proposes that corporatism in fact makes it a stronger and more autonomous state. Further, different criteria of autonomy are discussed. The public interest is explored through the concept of communicative action. Finally, the author tries to delimit the concept of autonomy in analysing some recent trends of development in Norwegian public policy.  相似文献   
97.
This article evaluates the new conception of symbols and rhetoric in organization theory (March and Olsen). It is a perspective that departs from the traditional instrumental view in political science (cf. Edelman}. This reorientation postulates the close connection between legitimacy and symbols, viewing symbolic language as a way of producing social integration. However, this perspective neglects the crucial aspect of legitimacy, i.e., a moral justification of power. Legitimacy concerns the cognitive and rational aspect of political argumentation rather than the expressive and symbolic aspect. Symbols, then, raise distinct analytical problems that refer to the authenticity and sincerity aspect of behaviour. Politics has to do with the just allocation of welfare, and symbols signify meaning and loyalty Thai governmental policy is merely symbolic, then, denotes that it does not produce any real effects.  相似文献   
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99.
Abstract. The article reviews policy-making patterns with respect to the importance and limits of sectorization and adds new analyses of the involvement of interest groups and bureaucrats in the legislative process. The pattern of sectoral policy-making appears to vary according to types of actors involved, stages of the process, party in government, and policy area in question. Basically however, it is a process of affected interests in action. While the findings fit well with some democratic principles they also give reason for some concern.  相似文献   
100.
In post‐modern society cultural cleavages are becoming at least as relevant as socio‐economic ones. They can only be properly researched by the survey method, which opens up a whole new area for both micro and macroanalysis. So, first, we pinpoint some of the main problems of research into value orientations. Second, we suggest a new analysis of attitudes towards gender and homosexuality, employing both micro and macro data. One key finding here is that attitudes towards gender and homosexuality are linked at the micro level, which explains some of the saliency of gender and homosexuality as a new emerging cleavage in advanced societies. At the macro level, the main finding is that attitudes towards gender and homosexuality are strongly embedded in cultural or civilisational differences among countries. The evidence suggests therefore that these attitudes do constitute a new cleavage in advanced societies.  相似文献   
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