Vision 2020: A View of Our Energy Future

The Morning Address was given by The Honorable George Allen. “The Regulatory Framework: Where Are We Headed?” session by Eric Finkbeiner, Senior Adviser for Policy, Office of Governor Robert McDonnell; David Christian, Chief Executive Officer, Dominion Generation; and Professor Joel Eisen, University of Richmond School of Law. Professor Noah Sachs, University of Richmond School of Law, served as moderator. “The Future of Coal” session by John Lain, Partner at McGuireWoods LLP; Cale Jaffe, Senior Attorney with the Southern Environmental Law Center; and W. Thomas Hudson, President of W. Thomas Hudson and Associates, Inc. and of the Virginia Coal Association. Stephen E. Taylor, Allen Chair Editor for the University of Richmond Law Review, served as moderator. “Nuclear Power: Is There a ‘Renaissance’?” session by Donald Irwin, Hunton & Williams; Christopher Paine, Director of Nuclear Program, Natural Resources Defense Council (invited); and Michael H. Montgomery, Vice President of Fuel Development, Lightbridge Corporation. Tricia Dunlap, Robert R. Merhige, Jr. Fellow at the University of Richmond School of Law, served as moderator. “Emerging Issues in Energy Policy” session by Mark Rosen, Deputy General Counsel, CNA Corporation; Jefferson Reynolds, Water Policy Director with the Virginia Department of Environmental Quality; Kruskaia Sierra-Escalante, Senior Counsel for the International Finance Corporation; and Edward Lowe, General Manager for Renewable Energy Market Development, GE Energy. Andrea W. Wortzel, Counsel with Hunton & Williams and Vice Chair of the Environmental Law Section of the Virginia State Bar, served as moderator. The Closing Address was given by The Honorable Carol M. Browner, Assistant to the President for Energy and Climate Change and Former Administrator of the Environmental Protection Agency (invited)

In-depth phenotyping for clinical stratification of Gaucher disease.

BackgroundThe Gaucher Investigative Therapy Evaluation is a national clinical cohort of 250 patients aged 5-87 years with Gaucher disease in the United Kingdom-an ultra-rare genetic disorder. To inform clinical decision-making and improve pathophysiological understanding, we characterized the course of Gaucher disease and explored the influence of costly innovative medication and other interventions. Retrospective and prospective clinical, laboratory and radiological information including molecular analysis of the GBA1 gene and comprising > 2500 variables were collected systematically into a relational database with banking of collated biological samples in a central bioresource. Data for deep phenotyping and life-quality evaluation, including skeletal, visceral, haematological and neurological manifestations were recorded for a median of 17.3 years; the skeletal and neurological manifestations are the main focus of this study.ResultsAt baseline, 223 of the 250 patients were classified as type 1 Gaucher disease. Skeletal manifestations occurred in most patients in the cohort (131 of 201 specifically reported bone pain). Symptomatic osteonecrosis and fragility fractures occurred respectively in 76 and 37 of all 250 patients and the first osseous events occurred significantly earlier in those with neuronopathic disease. Intensive phenotyping in a subgroup of 40 patients originally considered to have only systemic features, revealed neurological involvement in 18: two had Parkinson disease and 16 had clinical signs compatible with neuronopathic Gaucher disease-indicating a greater than expected prevalence of neurological features. Analysis of longitudinal real-world data enabled Gaucher disease to be stratified with respect to advanced therapies and splenectomy. Splenectomy was associated with an increased hazard of fragility fractures, in addition to osteonecrosis and orthopaedic surgery; there were marked gender differences in fracture risk over time since splenectomy. Skeletal disease was a heavy burden of illness, especially where access to specific therapy was delayed and in patients requiring orthopaedic surgery.ConclusionGaucher disease has been explored using real-world data obtained in an era of therapeutic transformation. Introduction of advanced therapies and repeated longitudinal measures enabled this heterogeneous condition to be stratified into obvious clinical endotypes. The study reveals diverse and changing phenotypic manifestations with systemic, skeletal and neurological disease as inter-related sources of disability

In-depth phenotyping for clinical stratification of Gaucher disease

Abstract: Background: The Gaucher Investigative Therapy Evaluation is a national clinical cohort of 250 patients aged 5–87 years with Gaucher disease in the United Kingdom—an ultra-rare genetic disorder. To inform clinical decision-making and improve pathophysiological understanding, we characterized the course of Gaucher disease and explored the influence of costly innovative medication and other interventions. Retrospective and prospective clinical, laboratory and radiological information including molecular analysis of the GBA1 gene and comprising > 2500 variables were collected systematically into a relational database with banking of collated biological samples in a central bioresource. Data for deep phenotyping and life-quality evaluation, including skeletal, visceral, haematological and neurological manifestations were recorded for a median of 17.3 years; the skeletal and neurological manifestations are the main focus of this study. Results: At baseline, 223 of the 250 patients were classified as type 1 Gaucher disease. Skeletal manifestations occurred in most patients in the cohort (131 of 201 specifically reported bone pain). Symptomatic osteonecrosis and fragility fractures occurred respectively in 76 and 37 of all 250 patients and the first osseous events occurred significantly earlier in those with neuronopathic disease. Intensive phenotyping in a subgroup of 40 patients originally considered to have only systemic features, revealed neurological involvement in 18: two had Parkinson disease and 16 had clinical signs compatible with neuronopathic Gaucher disease—indicating a greater than expected prevalence of neurological features. Analysis of longitudinal real-world data enabled Gaucher disease to be stratified with respect to advanced therapies and splenectomy. Splenectomy was associated with an increased hazard of fragility fractures, in addition to osteonecrosis and orthopaedic surgery; there were marked gender differences in fracture risk over time since splenectomy. Skeletal disease was a heavy burden of illness, especially where access to specific therapy was delayed and in patients requiring orthopaedic surgery. Conclusion: Gaucher disease has been explored using real-world data obtained in an era of therapeutic transformation. Introduction of advanced therapies and repeated longitudinal measures enabled this heterogeneous condition to be stratified into obvious clinical endotypes. The study reveals diverse and changing phenotypic manifestations with systemic, skeletal and neurological disease as inter-related sources of disability

Pour une histoire du risque

L’histoire du risque que propose ce livre déborde largement les notions de statistique, de calcul probabilitaire et de traitement assurantiel des dangers. Si, en effet, le risque a une histoire, le risque est aussi histoire, car il concerne le rapport des sociétés au temps. Tout rapport au risque tente, à partir de l’expérience passée, de saisir un avenir probable pour agir dans le présent. Chaque contexte, chaque époque, chaque territoire, chaque communauté appréhende les dangers selon ses ressources culturelles d’une part, et selon les enjeux politiques, sociaux et économiques qui la traversent d’autre part. Le risque est un fait de culture, reflétant la façon dont la société se représente elle-même, envisage les phénomènes qui la menacent et définit l’altérité qui la borne. Des historiens de tous horizons ont recours, dans ces pages, au concept de risque pour comprendre le passé, pour examiner leur objet de recherche sous un angle différent, qu’il s’agisse d’histoire des sciences et techniques ou du droit, ou d’histoire environnementale, sociale ou politique. Cette démarche commune dévoile des convergences insoupçonnées et permet aux auteurs de renouer avec un problème d’une intelligibilité historique globale, problème crucial qui a pourtant été abandonné par la très grande majorité des historiens au cours des dernières décennies

Exploring the role of ectomycorrhizal fungi in soil carbon dynamics

The extent to which ectomycorrhizal (ECM) fungi enable plants to access organic nitrogen (N) bound in soil organic matter (SOM) and transfer this growth-limiting nutrient to their plant host, has important implications for our understanding of plant–fungal interactions, and the cycling and storage of carbon (C) and N in terrestrial ecosystems. Empirical evidence currently supports a range of perspectives, suggesting that ECM vary in their ability to provide their host with N bound in SOM, and that this capacity can both positively and negatively influence soil C storage. To help resolve the multiplicity of observations, we gathered a group of researchers to explore the role of ECM fungi in soil C dynamics, and propose new directions that hold promise to resolve competing hypotheses and contrasting observations. In this Viewpoint, we summarize these deliberations and identify areas of inquiry that hold promise for increasing our understanding of these fundamental and widespread plant symbionts and their role in ecosystem-level biogeochemistry