Prenatal diagnosis of infantile GM 2 gangliosidosis type II (sandhoff disease) by detection of N-acetylglucosaminyl-oligosaccharides in amniotic fluid with high-performance liquid chromatography

Prenatal diagnosis of Sandhoff disease (infantile onset) at 16 weeks gestation has been made by detection and analysis of N-acetylglucosaminyl-oligosaccharides in amniotic fluid using high performance liquid chromatography. The elution profile for the branched chain oli-gosaccharides was identical with that obtained with neonatal and infantile Sandhoff urine. The concentration of the oligosaccharides in the fluid was 1/100th that of urine but when calculated relative to creatinine the levels were similar. No oligosaccharides were detected in normal control amniotic fluids (10 patients) at a similar gestational age. Based on the levels of the amniotic fluid oligosaccharides and the sensitivity limits of the assay, prenatal diagnosis of patients with the juvenile onset form of the disease may also be possible with this technique.     

CLIMATE CHANGE AND NATURAL HAZARDS IN NORTHERN CANADA: INTEGRATING INDIGENOUS PERSPECTIVES WITH GOVERNMENT POLICY

A study of the relationship between natural hazards and climate change in the international context provides the background for a discussion of the expected changes. In the context of this global discussion, this paper reviews the current perspectives of those natural hazards that are likely to be influenced by climate change, using northern Canada as a regional case study. The northern implications of the United Nations Framework Convention on Climate Change are examined, including the status of climate change action by the northern territorial governments, the evolving role of indigenous people, and the responsibility for climate change impacts. The difficulties surrounding natural hazards research in remote locations, and the approaches of indigenous people to natural hazards are then presented. The paper concludes with a suggested policy approach for climate change and natural hazards in northern Canada, underscoring the need for more comprehensive adaptive strategies to complement the current tendency to focus on the mitigation of greenhouse gases produced in this region.     

Thermal treatment and non-thermal technologies for remediation of manufactured gas plant sites

More than 1500 manufactured gas plant (MGP) sites exist throughout the U.S. Many are contaminated with coal tar from coal-fueled gas works which produced ‘town gas’ from the mid-1800s through the 1950s.^1,2 Virtually all old U.S. cities have such sites. Most are in downtown areas as they were installed for central distribution of manufactured gas. While a few sites are CERCLA/Superfund, most are not. However, the contaminants and methods used for remediation are similar to those used for Superfund clean-ups of coal tar contamination from wood-treating and coke oven facilities. Clean-up of sites is triggered by regulatory pressure, property transfers and re-development as well as releases to the environment — in particular, via groundwater migration. Due to utility de-regulation, site clean-ups may also be triggered by sale of a utility or of a specific utility site to other utilities. Utilities have used two approaches in dealing with their MGP sites. The first is ‘do nothing and hope for the best’. History suggests that, sooner or later, these sites become a bigger problem via a release, citizen lawsuit or regulatory/public service commission intervention. The second, far better approach is to define the problem now and make plans for waste treatment or immobilization. This paper describes recent experience with a high capacity/low cost thermal desorption process for this waste and reviews non-thermal technology, such as bio-treatment, capping, recycling, and dig and haul. Cost data is provided for all technologies, and a case study for thermal treatment is also presented.     

Industrial facility deactivation: A proactive approach for reducing costs and liabilities

For both private corporations and military branches, downsizing and consolidation are becoming more commonplace. A range of environmental concerns must be addressed to effectively implement a consolidation program. A facility deactivation program can often become a minefield for an organization. Responsible personnel are typically inexperienced with the process, and this can lead to costly mistakes. This article provides insight into this topic based on experience gained with a multifacility program and related environmental issues. It emphasizes the importance of detailed, up-front, proactive project planning; a solid program management system; and accurate identification of project objectives and deliverables.     

Focusing biodiversity research on the needs of decision makers

The project on Biodiversity Uncertainties and Research Needs (BURN) ensures the advancement of usable knowledge on biodiversity by obtaining input from decision makers on their priority information needs about biodiversity and then using this input to engage leading scientists in designing policy-relevant research. Decision makers articulated concerns related to four issues: significance of biodiversity; status and trends of biodiversity; management for biodiversity; and the linkage of social, cultural, economic, legal, and biological objectives. Leading natural and social scientists then identified the research required to address the decision makers' needs and determined the probability of success. The diverse group of experts reached consensus on several fundamental issues, helping to clarify the role of biodiversity in land and resource management. The BURN participants identified several features that should be incorporated into policy-relevant research plans and management strategies for biodiversity. Research and assessment efforts should be: multidisciplinary and integrative, participatory with stakeholder involvement, hierarchical (multiple scales), and problem- and region-specific. The activities should be focused regionally within a global perspective. Meta-analysis of existing data is needed on all fronts to assess the state of the science. More specifically, the scientists recommended six priority research areas that should be pursued to address the information needs articulated by decision makers: (1) characterization of biodiversity, (2) environmental valuation, (3) management for sustainability—for humans and the environment (adaptive management), (4) information management strategies, (5) governance and stewardship issues, and (6) communication and outreach. Broad recommendations were developed for each research area to provide direction for research planning and resource management strategies. The results will directly benefit those groups that require biodiversity research to address their needs—whether to develop policy, manage natural resources, or make other decisions affecting biodiversity.     

TEMPORAL AND GEOMORPHIC VARIATIONS OF STREAM STABILITY AND MORPHOLOGY: MAHOGANY CREEK,NEVADA1

ABSTRACT: Detailed studies of long-term management impacts on rangeland streams are few because of the cost of obtaining detailed data replicated in time. This study uses government agency aquatic habitat, stream morphologic, and ocular stability data to assess land management impacts over four years on three stream reaches of an important rangeland watershed in northwestern Nevada. Aquatic habitat improved as riparian vegetation reestablished itself with decreased and better controlled livestock grazing. However, sediment from livestock disturbances and road crossings and very low stream flows limited the rate of change. Stream type limited the change of pool variables and width/depth ratio, which are linked to gradient and entrenchment. Coarse woody debris removal due to previous management limited pool recovery. Various critical-element ocular stability estimates represented changes with time and differences among reaches very well. Ocular stability variables tracked the quantitative habitat and morphologic variables well enough to recommend that ocular surveys be used to monitor changes with time between more intensive aquatic surveys.     

INTEGRATED RIVER BASIN OPTIMIZATION: MODELING ECONOMIC AND HYDROLOGIC INTERDEPENDENCE1

ABSTRACT: This paper presents an integrated optimal control model that optimizes economic performance of reservoir management in watersheds in which there are significant economic and hydrologic interdependencies. The model is solved using the General Algebraic Modeling System (GAMS). Results show that application of this model to New Mexico's Rio Chama basin can increase total system benefits over historical benefits by exploiting complementarities between hydroelectricity production, instream recreation, and downstream lake recreation.