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1.
We report the finding of paternal isodisomy for chromosome 14 in a fetus found to have a der(14;14)(q10;q10) by amniocentesis. The pregnancy was complicated by severe polyhydramnios and elevated amniotic fluid alpha-fetoprotein (AFP). The infant showed features consistent with paternal uniparental disomy (UPD) including postnatal growth retardation, poor respiratory function, feeding difficulties, and evidence of hypertrophic cardiomyopathy. The present case, in addition to other reported cases of UPD involving balanced acrocentric rearrangements, supports testing for UPD in prenatally detected Robertsonian translocations and isochromosomes. Copyright © 2002 John Wiley & Sons, Ltd.  相似文献   
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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.  相似文献   
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Lake M?laren is the water supply and recreation area for more than 1 million people in central Sweden and subject to considerable environmental concern. To establish background data for assessments of contemporary levels of trophy and heavy metal pollution, sediment cores from the lake were analyzed. Diatom-inferred lake-water phosphorus concentrations suggest that pre-20th century nutrient levels in S?dra Bj?rkfj?rden, a basin in the eastern part of M?laren, were higher (c. 10-20 micrograms TP L-1) than previously assumed (c. 6 micrograms TP L-1). Stable lead isotope and lead concentration analyses from 3 basins (S. Bj?rkfj?rden, Gisselfj?rden and Ask?fj?rden) show that the lake was polluted in the 19th century and earlier from extensive metal production and processing in the catchment, particularly in the Bergslagen region. The lake has experienced a substantial improvement of the lead pollution situation in the 20th century following closure of the mining and metal industry. The lead pollution from the old mining industry was large compared to late-20th century pollution from car emissions, burning of fossil fuels and modern industries.  相似文献   
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Aichi Target 11 of the Convention on Biological Diversity urges, inter alia, that nations protect at least 17 % of their land, and that protection is effective and targets areas of importance for biodiversity. Five years before reporting on Aichi targets is due, we assessed the Philippines’ current protected area system for biodiversity coverage, appropriateness of management regimes and capacity to deliver protection. Although protected estate already covers 11 % of the Philippines’ land area, 64 % of its key biodiversity areas (KBAs) remain unprotected. Few protected areas have appropriate management and governance infrastructures, funding streams, management plans and capacity, and a serious mismatch exists between protected area land zonation regimes and conservation needs of key species. For the Philippines to meet the biodiversity coverage and management effectiveness elements of Aichi Target 11, protected area and KBA boundaries should be aligned, management systems reformed to pursue biodiversity-led targets and effective management capacity created.  相似文献   
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The spatial and temporal variability of transparency measured for 15 years at 7 stations near the San Diego sewage outfall has been investigated and compared to the temporal variability of sewage suspended solid discharge and flow rate. the purpose of the time-series analyses was to distinguish natural from human (sewage discharge) causes of temporal changes in transparency. the results show that: 1) variations in transparency are highly correlated over the entire area, but there is a gradient in means and variability in the direction perpendicular to the coast; 2) there are no long term trends for increase or decrease in the water clarity at any of the stations; 3) most of the variance of transparency is contained in the seasonal frequency band; 4) over the same time period sewage discharge has significantly increased and suspended solids decreased; 5) most of the variance of these human-caused properties is in the interannual frequency band; (6) there is no correlation at any time-lag between water clarity and suspended solid discharge or flow. These results lead to the conclusion that these anthropogenic properties are not affecting transparency, while natural factors such as seasonality and distance from coast do.  相似文献   
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Vehicular emissions of NO(x) and NH(3) result in elevated concentrations of nitrogen at roadside verges. To determine the extent that vehicular nitrogen emissions, disturbance and management affect the vegetation composition of road verges, a survey of 92 verges in Scotland was carried out with sites stratified by background nitrogen deposition and road type. NO(x) and NH(3) concentrations were monitored at 15 key sites for a year, and showed a decreasing gradient with increasing distance from the road. Ellenberg fertility indices of the vegetation communities also showed a general decrease with increasing distance from the road, but there was no straightforward correlation with NO(x) and NH(3) air concentrations between sites. Cover of bare ground, ruderal species and salt-tolerant species were highest at the verge edge. The proximity of the verge to traffic is important both in terms of NO(x) and NH(3) gradients, but also for deposited salt, grit and physical disturbance.  相似文献   
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The lack of scientific baseline information hinders appropriate design and management of protected areas. To illustrate the value of science to management, we consider five scenarios for the 202.0 km² Puerto Princesa Subterranean River National Park, Philippines: (1) closure to human activities, (2) and (3) two levels of increase in unplanned human activities, (4) creation of a forest corridor and (5) additional allocation of land for permanent or shifting agriculture. We then use habitat-specific bird density estimates to simulate the net effect of each scenario on 18 focal bird populations. Closure has significant benefits—populations of five species are predicted to increase by >50 % and nine by >25 %, but two secondary forest flycatchers, including the endemic and ‘Vulnerable’ Palawan flycatcher, decline dramatically, while the creation of a 4.0 km² forest corridor yields average increases across species of 2 ± 4 % (SD). In contrast, heavier unplanned park usage produces declines in all but a few species, while the negative effects of an extra 2.0 km² of shifting cultivation are 3–5 times higher than for a similar area of permanent agriculture and affect species whose densities are highest in primary habitats. Relatively small changes within the park, especially those associated with agricultural expansion, has serious predicted implications for local bird populations. Our models do not take into account the full complexities of bird ecology at a site, but they do provide park managers with an evidence base from which to make better decisions relating to biodiversity conservation obligations which their parks are intended to meet.  相似文献   
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Effective conservation management interventions must combat threats and deliver benefits at costs that can be achieved within limited budgets. Considerable effort has focused on measuring the potential benefits of conservation interventions, but explicit quantification of the financial costs of implementation is rare. Even when costs have been quantified, haphazard and inconsistent reporting means published values are difficult to interpret. This reporting deficiency hinders progress toward a collective understanding of the financial costs of management interventions across projects and thus limits the ability to identify efficient solutions to conservation problems or attract adequate funding. We devised a standardized approach to describing financial costs reported for conservation interventions. The standards call for researchers and practitioners to describe the objective and outcome, context and methods, and scale of costed interventions, and to state which categories of costs are included and the currency and date for reported costs. These standards aim to provide enough contextual information that readers and future users can interpret the cost data appropriately. We suggest these standards be adopted by major conservation organizations, conservation science institutions, and journals so that cost reporting is comparable among studies. This would support shared learning and enhance the ability to identify and perform cost‐effective conservation.  相似文献   
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