Management and organizational assessments: a review of selected organizations

This report is part of a larger project designed to assist the NRC in its responsibilities for assessing the management and organization of utilities applying for an operating license for a nuclear power plant. This report reviews the processes and criteria used by other organizations that conduct management and organization audits and evaluations. It was undertaken in order to provide data and a basis for future analysis by taking a comparative perspective. When considering changes in criteria and procedures as the NRC is doing, a standard benchmark is the performance of other organizations that are similarly situated. It was our goal to directly inform the NRC about the activities of other organizations so that a reconsideration of NRC activities could benefit from the perspective of organizations with a longer, broader, and different experience than the NRC has in the management and organization area. Data collected for this report has provided useful information in designing organization and administration guidelines and assessment procedures for consideration by the NRC

Is it possible to improve neurodevelopmental abnormalities in Down syndrome?

Down syndrome (DS) is a genetic pathology caused by the triplication of human chromosome 21. Although individuals with DS have various medical problems, intellectual disability is the most invalidating aspect of the pathology. Despite numerous efforts, the mechanisms whereby gene triplication leads to the DS phenotype have not been elucidated and there are, at present, no therapies to rescue brain developmental alterations and mental disability in individuals with DS. In this review, we focused on the major defects of the DS brain, comparing data regarding humans with DS and mouse models for DS, and therapeutic interventions attempted on animal DS models. Based on the promising results of pharmacotherapies in these models, we believe that it is possible to conclude that tools to improve brain development in DS are now almost at hand. We now know that it is possible to rescue and/or improve neurogenesis, neuron maturation, connectivity, neurodegeneration and behavior. We believe that the knowledge gained in DS mouse models provides a rational basis to start new clinical trials in infants, children and adults with DS, exploiting drugs that have proved able to rescue various facets of the DS neurologic phenotype. It is not unreasonable to consider that the results of these trials may provide a positive answer to the question: 'Is it possible to improve brain development in DS?'