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Arthrogryposis multiplex congenita (AMC) refers to an aetiologically heterogenous condition, which consists of joint contractures affecting two or more joints starting prenatally. The incidence is approximately one in 3000 live births; however, the prenatal incidence is higher, indicating a high intrauterine mortality. Over 320 genes have been implicated showing the genetic heterogeneity of the condition. AMC can be of extrinsic aetiology resulting from intrauterine crowding secondary to congenital structural uterine abnormalities (eg, bicornuate or septate uterus), uterine tumors (eg, fibroid), or multifetal pregnancy or intrinsic/primary/fetal aetiology, due to functional abnormalities in the brain, spinal cord, peripheral nerves, neuromuscular junction, muscles, bones, restrictive dermopathies, tendons and joints. Unlike many of the intrinsic/primary/fetal causes which are difficult to treat, secondary AMC can be treated by physiotherapy with good response. Primary cases may present prenatally with fetal akinesia associated with joint contractures and occasionally brain abnormalities, decreased muscle bulk, polyhydramnios, and nonvertex presentation while the secondary cases usually present with isolated contractures. Complete prenatal and postnatal investigations are needed to identify an underlying aetiology and provide information regarding its prognosis and inheritance, which is critical for the obstetrical care providers and families to optimize the pregnancy management and address future reproductive plans. 相似文献
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Type A behavior dimensions and optimism were examined as predictors of health and performance. In addition, this research also explored the ways that Type As and optimists cope with stressful situations. The achievement striving dimension of the Type A behavior pattern and optimism were positively related to class performance, while the anger/hostility dimension was positively related to the health symptom of anxiety. Optimism, on the other hand, was negatively related to anxiety. The interaction of achievement striving and optimism was negatively related to anxiety. Additionally, while achievement striving was positively related to problem-focused coping, irritability showed a negative association with problem-focused coping strategy. The results of this study provide insights for both individuals and organizations regarding how to cope with daily stresses in order to lower health risks and improve performance. 相似文献
187.
Susan Power Bratton Raymond C. Mathews Jr. Peter S. White 《Environmental management》1980,4(5):433-448
Agricultural management in Cades Cove, an historic district in Great Smoky Mountains National Park, has affected natural resources both within the district and in the adjoining natural areas. Aquatic impacts of haying and cattle grazing included increases in water temperatures, turbidity, nutrient loading, and bacterial counts and decreases in benthic macroinvertebrate density and fish biomass. Wildlife populations, including groundhogs, wild turkeys, and white-tailed deer, have increased in the open fields and around the periphery of the historic district. Intensive deer foraging has removed deciduous seedlings and saplings from woodlots, lowering species diversity and favoring coniferous reproduction. Cades Cove has limestone habitats unique in the park, and both deer browse and cattle grazing may have disturbed populations of rare plant species. Effects on water quality are detectable at a campground 15 stream km from the agricultural area, and the effects of deer foraging extend about 1 km beyond the open fields.Since historic landscape preservation is presently a goal of the park, managing for open vistas in Cades Cove will require some sort of continuing disturbance. Conversion of cattle pastures to hayfields would reduce aquatic impacts but the deer herd might increase as a result of reduced competition for forage. Retarding old field succession would increase populations of native plant species dependent on sunlight, but would require government-funded mowing. Other options are discussed. Completely eliminating the effects of the historic district on adjoining areas may be impossible, at least under present economic constraints. 相似文献
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Evaluating public participation in environmental decision-making: EPA's superfund community involvement program 总被引:1,自引:0,他引:1
This article discusses an 8-year, ongoing project that evaluates the Environmental Protection Agency's Superfund community involvement program. The project originated as a response to the Government Performance and Results Act, which requires federal agencies to articulate program goals, and evaluate and report their progress in meeting those goals. The evaluation project assesses how effective the Superfund community involvement program is in promoting public participation in decisions about how to clean up hazardous wastes at Superfund sites. We do three things in the article: (1) share our experience with evaluating an Agency public participation program, including lessons learned about methods of evaluation; (2) report evaluation results; and (3) address a number of issues pertaining to the evaluation of public participation in environmental decision-making. Our goal is to encourage more environmental managers to incorporate evaluation into their public participation programs as a tool for improving them. We found that written mail surveys were an effective and economical tool for obtaining feedback on EPA's community involvement program at Superfund sites. The evaluation focused on four criteria: citizen satisfaction with EPA information about the Superfund site, citizen understanding of environmental and human health risks associated with the site, citizen satisfaction with opportunities provided by EPA for community input, and citizen satisfaction with EPA's response to community input. While the evaluation results were mixed, in general, community members who were most informed about and involved in the cleanup process at Superfund sites generally were also the most satisfied with the community involvement process, and the job that EPA was doing cleaning up the site. We conclude that systematic evaluation provides meaningful and useful information that agencies can use to improve their public participation programs. However, there need to be institutionalized processes that ensure evaluation results are used to develop and implement strategies for improvement. 相似文献
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Luanne Y. Steffy Angela L. McGinty Claire Welty Susan S. Kilham 《Journal of the American Water Resources Association》2004,40(5):1269-1275
ABSTRACT: Valley Creek watershed is a small stream system that feeds the Schuylkill River near Philadelphia, Pennsylvania. The watershed is highly urbanized, including over 17 percent impervious surface cover (ISC) by area. Imperviousness in a watershed has been linked to fish community structure and integrity. Generally, above 10 to 12 percent ISC there is marked decline in fish assemblages with fish being absent above 25 percent ISC. This study quantifies the importance of ground water in maintaining fish species diversity in subbasins with over 30 percent ISC. Valley Creek contains an atypical fish assemblage in that the majority of the fish are warm‐water species, and the stream supports naturally reproducing brown trout, which were introduced and stocked from the early 1900s to 1985. Fish communities were quantified at 13 stations throughout the watershed, and Simpson's species diversity index was calculated. One hundred and nine springs were located, and their flow rates measured. A cross covariance analysis between Simpson's species diversity index and spring flow rates upstream of fish stations was performed to quantify the spatial correlation between these two variables. The correlation was found to be highest at lag distances up to about 400 m and drop off significantly beyond lag distances of about 800 m. 相似文献
190.
Cesium migration in Hanford sediment: a multisite cation exchange model based on laboratory transport experiments 总被引:1,自引:0,他引:1
Cs+ transport experiments carried out in columns packed with uncontaminated Hanford formation sediment from the SX tank farm provide strong support for the use of a multisite, multicomponent cation exchange model to describe Cs+ migration in the Hanford vadose zone. The experimental results indicate a strong dependence of the effective Cs+ Kd on the concentrations of other cations, including Na+ that is present at high to extremely high concentrations in fluids leaking from the Hanford SX tanks. A strong dependence of the Cs+ Kd on the aqueous Cs+ concentration is also apparent, with retardation of Cs+ increasing from a value of 41 at a Cs+ concentration of 10(-4) M in the feed solution to as much as 282 at a Cs+ concentration of 5x10(-7) M, all in a background of 1 M NaNO3. The total cation exchange capacity (CEC) of the Hanford sediment was determined using 22Na isotopic equilibrium exchange in a flow-through column experiment. The value for the CEC of 120 microeq/g determined with this method is compatible with a value of 121.9 microeq/g determined by multi-cation elution. While two distinct exchange sites were proposed by Zachara et al. [Geochim. Cosmochim. Acta 66 (2002) 193] based on binary batch exchange experiments, a third site is proposed in this study to improve the fit of the Cs+-Na+ and Cs+-Ca+ exchange data and to capture self-sharpened Cs+ breakthrough curves at low concentrations of Cs+. Two of the proposed exchange sites represent frayed edge sites (FES) on weathered micas and constitute 0.02% and 0.22% of the total CEC. Both of the FES show a very strong selectivity for Cs+ over Na+ (K(Na-Cs)=10(7.22) and 10(4.93), respectively). The third site, accounting for over 99% of the total CEC, is associated with planar sites on expansible clays and shows a smaller Na+-Cs+ selectivity coefficient of 10(1.99). Parameters derived from a fit of binary batch experiments alone tend to under predict Cs+ retardation in the column experiments. The transport experiments indicate 72-90% of the Cs+ sorbed in experiments targeting exchange on FES was desorbed over a 10- and 24-day period, respectively. At high Cs+ concentrations, where sorption is controlled primarily by exchange on planar sites, 95% of the Cs+ desorption was desorbed. Most of the difficulty in desorbing Cs+ from FES is a result of the extremely high selectivity of these sites for Cs+, although truly irreversible sorption as high as 23% was suggested in one experiment. The conclusion that Cs+ exchange is largely reversible in a thermodynamic sense is supported by the ability to match Cs+ desorption curves almost quantitatively with an equilibrium reactive transport simulation. The model for Cs+ retardation developed here qualitatively explains the behavior of Cs+ in the Hanford vadose zone underneath a variety of leaking tanks with differing salt concentrations. The high selectivity of FES for Cs+ implies that future desorption and migration is very unlikely to occur under natural recharge conditions. 相似文献