Cohort Profile: Pregnancy And Childhood Epigenetics (PACE) Consortium.

Development Psychopathology in context: famil

Method to develop data supporting consequence analyses of transporting nuclear materials in the United States

The Transportation System Safety Evaluation (TSSE) program at Sandia National Laboratories' Transportation Technology Center was initiated to provide the necessary information on source terms for nuclear materials subjected to extreme environments. The techniques for derivation of source terms for the fuel alone has been described as well as the outline for package response. An additional facet of this problem is the development of analytical methods to describe the transport of the released radionuclides from the fuel rods to possible release points. This work is also covered in the TSSE program. Not all the work required will be performed or funded by Sandia; rather existing work will be sought out and ongoing work will be utilized in an attempt to unify the presentation of data and thus increase its usefulness

Relative consequences of transporting hazardous materials

The objective of this paper is to discuss methods under study at Transportation Technology Center to develop a perspective on how technical measures of hazard and risk relate to perception of hazards, harm, and risks associated with transporting hazardous materials. This paper is concerned with two major aspects of the relative hazards problem. The first aspect is the analyses of the possible effects associated with exposure to hazardous materials as contained in the following two parts: outlines of possible problems and controversies that could be encountered in the evaluation and comparisons of hazards and risks; and description of the various measures of harm (hazards or dangers) and subsequent comparisons thereof. The second aspect of this paper leads into a presentation of the results of a study which had the following purposes: to develop analytical techniques for a consistent treatment of the phenomenology of the consequences of a release of hazardous materials; to reduce the number of variables in the consequence analyses by development of transportation accident scenarios which have the same meteorological conditions, demography, traffic and population densities, geographical features and other appropriate conditions and to develop consistent methods for presenting the results of studies and analyses that describe the phenomenology and compare hazards. The results of the study are intended to provide a bridge between analytical certainty and perception of the hazards involved. Understanding the differences in perception of hazards resulting from transport of various hazardous materials is fraught with difficulties in isolating the qualitative and quantitative features of the problem. By relating the quantitative impacts of material hazards under identical conditions, it is hoped that the perceived differences in material hazards can be delineated and evaluated

New NBIA subtype: Genetic, clinical, pathologic, and radiographic features of MPAN

Objective: to assess the frequency of mutations in C19orf12 in the greater neurodegeneration with brain iron accumulation (NBIA) population and further characterize the associated phenotype.Methods: samples from 161 individuals with idiopathic NBIA were screened, and C19orf12 mutations were identified in 23 subjects. Direct examinations were completed on 8 of these individuals, and medical records were reviewed on all 23. Histochemical and immunohistochemical studies were performed on brain tissue from one deceased subject.Results: a variety of mutations were detected in this cohort, in addition to the Eastern European founder mutation described previously. The characteristic clinical features of mitochondrial membrane protein-associated neurodegeneration (MPAN) across all age groups include cognitive decline progressing to dementia, prominent neuropsychiatric abnormalities, and a motor neuronopathy. A distinctive pattern of brain iron accumulation is universal. Neuropathologic studies revealed neuronal loss, widespread iron deposits, and eosinophilic spheroidal structures in the basal ganglia. Lewy neurites were present in the globus pallidus, and Lewy bodies and neurites were widespread in other areas of the corpus striatum and midbrain structures.Conclusions: MPAN is caused by mutations in C19orf12 leading to NBIA and prominent, widespread Lewy body pathology. The clinical phenotype is recognizable and distinctive, and joins pantothenate kinase-associated neurodegeneration and PLA2G6-associated neurodegeneration as one of the major forms of NBI