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1.
M. Peter Marinkovich MD Guerrino Meneguzzi Robert E. Burgeson Claudine Blanchet-Bardon Karen A. Holbrook Lynne T. Smith Angela M. Christiano Jean-Paul Ortonne 《黑龙江环境通报》1995,15(11):1027-1034
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.
Michael Greenberg Joanna Burger Michael Gochfeld David Kosson Karen Lowrie Henry Mayer Charles W. Powers Conrad D. Volz Vikram Vyas 《补救:环境净化治理成本、技术与工艺杂志》2005,16(1):91-105
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.
8.
Boundaries and Corridors as a Continuum of Ecological Flow Control: Lessons from Rivers and Streams 总被引:4,自引:0,他引:4
Abstract: Landscape boundaries and corridors are areas of small spatial extent relative to their large effects on ecological flows. The trend in ecological literature is to treat corridors and boundaries as separate phenomena on the landscape. This approach, however, misses a fundamental aspect they have in common: their strong influence on ecological flows. Corridors and boundaries exist at opposite ends of a permeability gradient, differing in their effects on rates and direction of flow. The position of landscape structures along this permeability gradient depends on attributes of both the flow and of the structure itself. We discuss boundaries and corridors in terms of mover specificity, scale, and effects on different levels of ecological organization, using rivers and streams to illustrate our points. We predict which structures will act as boundaries or corridors and at what spatial and temporal scales they are likely to be relevant. Considering the function of landscape structures across the boundary-corridor continuum will provide researchers and managers with a more complete, holistic viewpoint and will allow better strategies to attain conservation goals. 相似文献
9.
The migration of biogeochemical gradients is a useful framework for understanding the evolution of biogeochemical conditions in groundwater at waste sites contaminated with metals and radionuclides. This understanding is critical to selecting sustainable remedies and evaluating sites for monitored natural attenuation, because most attenuation mechanisms are sensitive to geochemical conditions, such as pH and redox potential. Knowledge of how gradients in these parameters evolve provides insights into the behavior of contaminants with time and guides characterization, remedy selection, and monitoring efforts. An example is a seepage basin site at the Savannah River Site in South Carolina where low‐level acidic waste has seeped into groundwater. The remediation of this site relies, in part, on restoring the natural pH of the aquifer by injecting alkaline solutions. The remediation will continue until the pH upflow of the treatment zone increases to an acceptable value. The time required to achieve this objective depends on the time it takes the trailing pH gradient, the gradient separating the plume from influxing natural groundwater, to reach the treatment zone. Predictions of this length of time will strongly influence long‐term remedial decisions. © 2008 Wiley Periodicals, Inc. 相似文献
10.