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151.
Very-long-chain acyl-coenzyme A (CoA) dehydrogenase deficiency (VLCADD) (OMIM #201475) is an autosomal recessive disorder of fatty acid oxidation. Major phenotypic expressions are hypoketotic hypoglycemia, hepatomegaly, cardiomyopathy, myopathy, rhabdomyolysis, elevated creatinine kinase, and lipid infiltration of liver and muscle. At the same time, it is a rare cause of Sudden Infant Death Syndrome (SIDS) or unexplained death in the neonatal period [1-4]. We report a patient with VLCADD whose parents were investigated for infanticide because her three previous siblings had suddenly died after normal deliveries.  相似文献   
152.
Based on fieldwork in the Kitgum district of northern Uganda, this paper investigates the experience of justice for the large number of survivors, female and male, of sexual violence as a result of the Lord's Resistance Army (LRA) insurgency and its aftermath. It asks: what justice do survivors of sexual violence want; what access to justice do survivors of LRA sexual violence have; what access to justice do the survivors of current sexual violence have; and what are the problems faced with delivering justice. It finds that the response has been minimal, ineffective and inappropriate. It concludes that the neglect of justice has serious implications for the attitudes on Acholi people towards the current Uganda government, for it perpetuates the perceived sense of marginalisation felt by many Acholi and which was a contributor to the LRA rebellion.  相似文献   
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Derrick Bell’s work challenges the dichotomy that separates legitimate legal reasoning from “mere” fiction through hybrids that play across science fiction, Platonic dialogue, and autobiography. Despite its merits, I argue that Bell’s position reifies and strengthens, rather than deconstructs, structures of tyranny; it maintains the problematic rhetorical construction of United States race relations in terms of the black–white divide, either alienating, or leaving little or no room for other racial groups constructively to revise power and identity. In contrast, bell hooks’, Mari Matsuda’s and Robert Chang’s work, though tentatively sketched, construes current race and gender relations in terms of a chiaroscuro of power, rather than a simple dichotomy.
Caroline Joan (Kay) PicartEmail:
  相似文献   
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The rise of virtualisation and cloud computing is one of the most significant features of computing in the last 10 years. However, despite its popularity, there are still a number of technical barriers that prevent it from becoming the truly ubiquitous service it has the potential to be. Central to this are the issues of data security and the lack of trust that users have in relying on cloud services to provide the foundation of their IT infrastructure. This is a highly complex issue, which covers multiple inter-related factors such as platform integrity, robust service guarantees, data and network security, and many others that have yet to be overcome in a meaningful way. This paper presents a concept for an innovative integrated platform to reinforce the integrity and security of cloud services and we apply this in the context of Critical Infrastructures to identify the core requirements, components and features of this infrastructure.  相似文献   
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