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1.
Herlitz junctional epidermolysis bullosa (HJEB) is a severe blistering disorder which usually results in death during infancy. We have previously shown that the anchoring filament protein laminin-5 (kalinin/nicein), which mediates keratinocyte attachment and dermal–epidermal cohesion, is abnormally expressed in individuals with HJEB. Laminin-5 was detected by Western blot analysis in amniotic fluid from 44 consecutive normal secondtrimester control pregnancies, but was undetectable in second-trimester amniotic fluid from four pregnancies with fetuses affected by HJEB. In one case of severe non-Herlitz JEB, laminin-5 was detected in both amniotic fluid and skin. In human amniotic fluid, the laminin-5 a3 subunit was processed to a major 165 kD species and a minor 145 kD species and the β2 subunit was partially processed to 105 kD. Although laminin-5 was covalently associated with laminin-6 (K-laminin) in amniotic membrane, no covalent interaction was detected in amniotic fluid. Laminin-5 from amniotic fluid strongly supported keratinocyte attachment. These results suggest that Western blot analysis of second-trimester amniotic fluid is useful in determining the prenatal diagnosis of HJEB and that laminin-5 may serve a physiologically important function in amniotic fluid.  相似文献   
2.
The karyotype of cultured amniotic fluid cells obtained on the indication of advanced maternal age was shown to be a mosaic 45,X/46,X,r(?). The small size and banding pattern made it difficult to determine whether the ring was derived from and X or a Y chromosome, or even from an autosome. By using an X-centromeric probe and fluorescence in situ hybridization (FISH), we demonstrated the ring to have an X centromere. Thus, a more complete genetic counselling was possible. This confirms the usefulness of FISH in identifying and characterizing this and other chromosome rearrangements in prenatal diagnosis.  相似文献   
3.
We present a case of fetal abdominal cystic hygroma that presented at 19 weeks of gestation. Ultrasonographic evaluation of the fetus revealed soft tissue enlargement of the left leg and a retroperitoneal mass in the left pelvis and abdomen. This represents the first reported case of prenatal diagnosis of abdominal cystic hygroma.  相似文献   
4.
This study investigates the potential for perchlorate biodegradation in the sediments of the Las Vegas Wash area in Henderson, Nevada. The continuous transport of perchlorate from a contaminated seepage to the Las Vegas Wash, Lake Mead, and the Colorado River has resulted in considerable deposition of perchlorate along the sediments of the Las Vegas Wash. The contaminated sediments act as a distributed source of perchlorate, making efforts to stop the flow of perchlorate to the Colorado River very challenging. In this study, perchlorate‐ reducing bacteria were enumerated and microcosm tests were performed to investigate the role of indigenous microorganisms and the limitations to natural perchlorate biodegradation in the contaminated sediments. The results of microcosm tests revealed that, despite the high number of perchloratereducing bacteria present, natural perchlorate in the area appears to be limited by (1) high salinity levels, the presence of nitrate, and the low perchlorate concentrations present in the sediments and (2) an insufficient carbon source. However, the potential for in situ bioremediation of the sediments along the Wash area is considered to be high due to the presence of significant numbers of perchlorate‐ reducing bacteria and to the ease in which an additional carbon source could be provided to sustain nitrate and perchlorate biodegradation. The economics of this process are expected to be very favorable; however, detailed cost estimates, pilot‐scale testing, and permit applications are required before this concept can be applied. © 2005 Wiley Periodicals, Inc.  相似文献   
5.
This article discusses creating a sustainably protective engineered and human management system in perpetuity for sites with long‐lived radiological and chemical hazards. This is essential at this time because the federal government is evaluating its property as assets and attempting to reduce its holdings, while seeking to assure that health and ecosystems are not put at risk. To assist those who have a stake in the remediation, management, and stewardship of these and analogous privately owned sites, this article discusses current end‐state planning by reviewing the federal government's accelerated efforts to reduce its footprint and how those efforts relate to sustainability. The article also provides a list of questions organized around six elements of risk management and primary, secondary, and tertiary disease and injury prevention. Throughout the article, the U.S. Department of Energy (DOE) is used as an example of an organization that seeks to reduce its footprint, manage its budget, and be a steward of the sites that it is responsible for. However, the approach and questions are appropriate for land controlled by the Department of Defense (DOD), the General Services Administration (GSA), and other public and private owners of sites with residual contamination. © 2005 Wiley Periodicals, Inc.  相似文献   
6.
In the UK, the Environmental Quality Standard for manganese has recently been lowered to 30 microg/L (annual average), which is less than the UK Drinking Water Inspectorate's Maximum Permitted Concentration Value (50 microg/L). Current passive treatment systems for manganese removal operate as open-air gravel-bed filters, designed to maximize either influent light and/or dissolved oxygen. This requires large areas of land. A novel enhanced bioremediation treatment system for manganese removal has been developed that consists of a passively aerated subsurface gravel bed. The provision of air at depth and the use of catalytic substrates help overcome the slow kinetics usually associated with manganese oxidation. With a residence time of only 8 h and an influent manganese concentration of approximately 20 mg/L, >95% of the manganese was removed. The treatment system also operates successfully at temperatures as low as 4 degrees C and in total darkness. These observations have positive implications for manganese treatment using this technique in both colder climates and where large areas of land are unavailable. Furthermore, as the operation of this passive treatment system continually generates fresh manganese oxyhydroxide, which is a powerful sorbent for most pollutant metals, it potentially has major ancillary benefits as a removal process for other metals, such as zinc.  相似文献   
7.
Some speculate that bioaerosols from land application of biosolids pose occupational risks, but few studies have assessed aerosolization of microorganisms from biosolids or estimated occupational risks of infection. This study investigated levels of microorganisms in air immediately downwind of land application operations and estimated occupational risks from aerosolized microorganisms. In all, more than 300 air samples were collected downwind of biosolids application sites at various locations within the United States. Coliform bacteria, coliphages, and heterotrophic plate count (HPC) bacteria were enumerated from air and biosolids at each site. Concentrations of coliforms relative to Salmonella and concentrations of coliphage relative to enteroviruses in biosolids were used, in conjunction with levels of coliforms and coliphages measured in air during this study, to estimate exposure to Salmonella and enteroviruses in air. The HPC bacteria were ubiquitous in air near land application sites whether or not biosolids were being applied, and concentrations were positively correlated to windspeed. Coliform bacteria were detected only when biosolids were being applied to land or loaded into land applicators. Coliphages were detected in few air samples, and only when biosolids were being loaded into land applicators. In general, environmental parameters had little impact on concentrations of microorganisms in air immediately downwind of land application. The method of land application was most correlated to aerosolization. From this large body of data, the occupational risk of infection from bioaerosols was estimated to be 0.78 to 2.1%/yr. Extraordinary exposure scenarios carried an estimated annual risk of infection of up to 34%, with viruses posing the greatest threat. Risks from aerosolized microorganisms at biosolids land application sites appear to be lower than those at wastewater treatment plants, based on previously reported literature.  相似文献   
8.
Although considerable attention is devoted to environmental monitoring and assessment with respect to both pollutants and the status of particular plant or animal populations, less attention is devoted to assessing people's attitudes about the relative importance of ecological resources. In this paper we examine the attitudes and perceptions about ecological resources of people living around the Department of Energy's Savannah River Site (SRS), in South Carolina. Our overall hypothesis is that people who are directly affected by the possible outcomes and consequences of a particular hazard (i.e., those people employed at SRS) will undervalue the risks and overvalue the potential benefits from future land uses that favor continued site activity, compared to people who live near but are not employed at SRS. We interviewed 286 people attending the Aiken Trials horse show on 14 March 1997. There were few gender differences, although men hunted and fished more than women, women ranked three environmental concerns as more severe than did men, and women were more concerned about the effect of SRS on property values. Maintenance of SRS as a National Environmental Research Park ranked first as a future land use; nuclear production ranked second, followed by hunting and hiking. Only residential development ranked very low as a future land use. There were many differences as a function of employment history at SRS: 1) people who work at SRS think that the federal government should spend funds to clean up all nuclear facilities, and they think less money should be spent on other environmental problems than did non-employees, 2) people who work at SRS ranked continued current uses of SRS higher than did people who never worked at SRS, and 3) people who work at SRS are less concerned about the storage of nuclear material or accidents at the site than are people who never worked at the site.  相似文献   
9.
Environmental Science and Pollution Research - This work proposes a novel approach for the coupling of ozonation and Fenton processes using a new prototype of a high rotation bubble reactor (HRBR),...  相似文献   
10.
Environmental Science and Pollution Research - Nowadays, the use of different nanoscale structures has been introduced to a large number of research areas. One of these is the treatment and...  相似文献   
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