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1.
Stickler syndrome is an autosomal dominant disorder of the connective tissue which includes ocular and systemic manifestations. We report on a large kindred in which we were able to demonstrate very tight linkage between the disease and the type II collagen gene (COL2A1) (LOD score 3·91 at θ=0). In a family in which the father and one of his daughters were severely affected, DNA analysis from a chorionic villus sample demonstrated that the fetus possessed the normal allele of COL2A1. Thereafter a normal child was born. 相似文献
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The New Jersey Toxics Reduction Workplan for Ny-Nj Harbor: Distribution of Pcdd/Fs in Ambient Waters
As part of the NJ Toxics Reduction Workplan for NY-NJ Harbor, ambient water samples were collected at fifteen locations along
the tidal portions of the Hackensack, Passaic, Raritan, Rahway and Elizabeth Rivers, and in Newark Bay, the Arthur Kill, and
Kill van Kull. A Trace Organics Platform Sampler was used to collect a total of 75 suspended sediment phase samples between
June 2000 and May 2002. These samples were analyzed for spatial and wet vs. dry weather trends in the 17 polychlorinated dibenzo-p-dioxins
and dibenzofurans (PCDD/Fs; modified USEPA Method 1613b). Mean total PCDD/F (tPCDD/F) concentrations at the sampling locations
ranged between 3.8 and 41.5 ng/g. On average, OCDD accounted for almost 80% of the tPCDD/F concentrations; mean total [PCDD/F
– OCDD] concentrations ranged between 0.84 and 5.20 ng/g at the sampling locations. Mean 2,3,7,8-TCDD concentrations ranged
between 0.003 and 0.28 ng/g, with the highest concentrations (> 0.10 ng/g) along the tidal Passaic and lower Hackensack Rivers,
and in upper Newark Bay. Mean tPCDD/F Toxic Equivalency Quotients (TEQ) ranged between 45 and 344 TEQ pg/g, with the highest
levels found in the lower Passaic River. Toxicity was primarily driven by 2,3,7,8-TCDD concentrations in the Passaic and Hackensack
Rivers, and in Newark Bay. Examples of congener distribution patterns at some of the sampling locations are also presented. 相似文献
6.
K. Bruce Jones Daniel T. Heggem Timothy G. Wade Anne C. Neale Donald W. Ebert Maliha S. Nash Megan H. Mehaffey Karl A. Hermann Anthony R. Selle Scott Augustine Iris A. Goodman Joel Pedersen David Bolgrien J. Max Viger Dean Chiang Cindy J. Lin Yehong Zhong Joan Baker Rick D. Van Remortel 《Environmental monitoring and assessment》2000,64(1):227-245
The Environmental Monitoring and Assessment Program (EMAP) is proposing an ambitious agenda to assess the status of streams and estuaries in a 12-State area of the western United States by the end of 2003. Additionally, EMAP is proposing to access landscape conditions as they relate to stream and estuary conditions across the west. The goal of this landscape project is to develop a landscape model that can be used to identify the relative risks of streams and estuaries to potential declines due to watershed-scale, landscape conditions across the west. To do so, requires an understanding of quantitative relationships between landscape composition and pattern metrics and parameters of stream and estuary conditions. This paper describes a strategic approach for evaluating the degree to which landscape composition and pattern influence stream and estuary condition, and the development and implementation of a spatially-distributed, landscape analysis approach. 相似文献
7.
Laboratory leaching tests may be used for source term determination as a basis for risk assessment for soil-groundwater pathways on contaminated sites. In order to evaluate different leaching procedures, batch extraction tests and percolation tests were performed using three reference materials produced from contaminated soil, demolition waste and municipal solid waste incinerator bottom ash. Emphasis was placed on the investigation of the leachability of the heavy metals copper and chromium, polycyclic aromatic hydrocarbons (PAHs) and the anions chloride and sulfate. Significant discrepancies between column experiments and batch/extraction tests were found for the release of PAHs and to a lesser extent for the heavy metals Cu and Cr. Additionally interlaboratory comparisons were conducted based on different leaching tests with the reference materials and evaluated using the criteria of comparability and reproducibility. The best reproducibility was achieved for all investigated substances in column tests. The reproducibility of batch tests was acceptable except for PAHs. The results from the experimental work will help establish standardized and feasible laboratory procedures as fundamental for substance specific risk assessment of contaminated sites. 相似文献
8.
This research seeks for improved understanding regarding the interaction of meaningful work and the work–family interface. Existing literature suggests that experiencing a sense of calling toward work makes the work domain particularly salient to employees compared to other life domains. In this article, we draw on this idea, rooted in identity theory, to hypothesize that a sense of calling toward work diminishes the effects of work–family conflict and work–family enrichment on employee's job and life satisfaction. We test these ideas in two studies. First, we surveyed an alumni sample of 598 employees from various jobs, industries, and job levels. Then, in a constructive replication, we surveyed 327 employees using a time-lagged design. Calling was found to significantly buffer the effect of work–family conflict on job satisfaction in Study 2, but not Study 1. Calling did not buffer the effect of conflict on life satisfaction in either study. However, both studies demonstrated that calling attenuated (substituted for) the effect of work–family enrichment on job satisfaction. Study 1 supported the idea that calling attenuates the effect of enrichment on life satisfaction; however, this interactive effect was reversed in Study 2, contrary to expectations. We discuss implications for theory and practice related to callings and career choices, as well as for the role of calling and work identity in the work–family interface. 相似文献
9.
A bimolecular rate constant, kOH+Benzyl alcohol, of (28 ± 7) × 10?12 cm3 molecule?1 s?1 was measured using the relative rate technique for the reaction of the hydroxyl radical (OH) with benzyl alcohol, at (297 ± 3) K and 1 atm total pressure. Additionally, an upper limit of the bimolecular rate constant, kO3+Benzyl alcohol, of approximately 6 × 10?19 cm3 molecule?1 s?1 was determined by monitoring the decrease in benzyl alcohol concentration over time in an excess of ozone (O3). To more clearly define part of benzyl alcohol's indoor environment degradation mechanism, the products of the benzyl alcohol + OH were also investigated. The derivatizing agents O-(2,3,4,5,6-pentafluorobenzyl)hydroxylamine (PFBHA) and N,O-bis(trimethylsilyl) trifluoroacetamide (BSTFA) were used to positively identify benzaldehyde, glyoxal and 4-oxopentanal as benzyl alcohol/OH reaction products. The elucidation of other reaction products was facilitated by mass spectrometry of the derivatized reaction products coupled with plausible benzyl alcohol/OH reaction mechanisms based on previously published volatile organic compound/OH gas-phase reaction mechanisms. 相似文献
10.
Benjamin M. Jones Christopher D. Arp Kenneth M. Hinkel Richard A. Beck Joel A. Schmutz Barry Winston 《Environmental management》2009,43(6):1071-1084
Lakes are dominant landforms in the National Petroleum Reserve Alaska (NPRA) as well as important social and ecological resources.
Of recent importance is the management of these freshwater ecosystems because lakes deeper than maximum ice thickness provide
an important and often sole source of liquid water for aquatic biota, villages, and industry during winter. To better understand
seasonal and annual hydrodynamics in the context of lake morphometry, we analyzed lakes in two adjacent areas where winter
water use is expected to increase in the near future because of industrial expansion. Landsat Thematic Mapper and Enhanced
Thematic Mapper Plus imagery acquired between 1985 and 2007 were analyzed and compared with climate data to understand interannual
variability. Measured changes in lake area extent varied by 0.6% and were significantly correlated to total precipitation
in the preceding 12 months (p < 0.05). Using this relation, the modeled lake area extent from 1985 to 2007 showed no long-term trends. In addition, high-resolution
aerial photography, bathymetric surveys, water-level monitoring, and lake-ice thickness measurements and growth models were
used to better understand seasonal hydrodynamics, surface area-to-volume relations, winter water availability, and more permanent
changes related to geomorphic change. Together, these results describe how lakes vary seasonally and annually in two critical
areas of the NPRA and provide simple models to help better predict variation in lake-water supply. Our findings suggest that
both overestimation and underestimation of actual available winter water volume may occur regularly, and this understanding
may help better inform management strategies as future resource use expands in the NPRA. 相似文献