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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   
2.
On June 5 and 6 of 1980, two parallel plume oxidation studies were carried out in the vicinity of the Tennessee Valley Authority's Colbert Steam Plant. One study was performed in a smog chamber into which stack gases were injected and mixed with ambient air. The other study included direct airborne sampling of the power plant plume. Atmospheric oxidation rates for the conversion of SO2 to SO4 2- and the removal rates of NO x (which is presumably the rate of NO3 - formation) were estimated for both studies. The SO2 to SO4 2- rate coefficients were found to be 0.022 ± 0.009 h-1 for both chamber experiments and the first airborne sampling day. For the second day, a rate constant of 0.041 ± 0.052 h-1 was estimated from the aircraft data. The large deviation in this value is explained by the fact that the plume from the power plant combined and reacted with the urban plume from the city of Florence, AL. The formation of a very large "O3 bulge" on this day is also attributed to the mixed plumes. The first order rate coefficients for NO x removal were estimated to be 0.27 ± 0.14 h-1 for both chamber experiments and the first airborne sampling day. A NO x removal rate could not be determined for the second airborne sampling day.  相似文献   
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A comparison of air quality data obtained at a downtown roof top location, a surburban area, a main road curbside and adjacent to a major highway was conducted. The results of this study revealed that a reasonably good correlation between daily average concentrations at these locations can be established. For primary pollutants it was found that within the immediate release zone their average concentration is 3.5 to 4.0 times higher than at a close location but at roof top level. Total suspended particulate (TSP) concentration was found to increase by about 80%, and O3 is reduced by about 25%. No significant difference was obtained between daily averages obtained at the city center location and the suburb site 5 km away.  相似文献   
5.
To examine colloid transport in geochemically heterogeneous porous media at a scale comparable to field experiments, we monitored the migration of silica-coated zirconia colloids in a two-dimensional layered porous media containing sand coated to three different extents by ferric oxyhydroxides. Transport of the colloids was measured over 1.65 m and 95 days. Colloid transport was modeled by an advection-dispersion-deposition equation incorporating geochemical heterogeneity and colloid deposition dynamics (blocking). Geochemical heterogeneity was represented as favorable (ferric oxyhydroxide-coated) and unfavorable (uncoated sand) deposition surface areas. Blocking was modeled as random sequential adsorption (RSA). Release of deposited colloids was negligible. The time to colloid breakthrough after the onset of blocking increased with increasing ferric oxyhydroxide-coated surface area. As the ferric oxyhydroxide surface area increased, the concentration of colloids in the breakthrough decreased. Model-fits to the experimental data were made by inverse solutions to determine the fraction of surface area favorable for deposition and the deposition rate coefficients for the favorable (ferric oxyhydroxide-coated) and unfavorable sites. The favorable deposition rate coefficient was also calculated by colloid filtration theory. The model described the time to colloid breakthrough and the blocking effect reasonably well and estimated the favorable surface area fraction very well for the two layers with more than 1% ferric oxyhydroxide coating. If mica edges in the uncoated sand were considered as favorable surface area in addition to the ferric oxyhydroxide coatings, the model predicted the favorable surface area fraction accurately for the layer with less than 1% ferric oxyhydroxide coating.  相似文献   
6.
A two-dimensional model for virus transport in physically and geochemically heterogeneous subsurface porous media is presented. The model involves solution of the advection-dispersion equation, which additionally considers virus inactivation in the solution, as well as virus removal at the solid matrix surface due to attachment (deposition), release, and inactivation. Two surface inactivation models for the fate of attached inactive viruses and their subsequent role on virus attachment and release were considered. Geochemical heterogeneity, portrayed as patches of positively charged metal oxyhydroxide coatings on collector grain surfaces, and physical heterogeneity, portrayed as spatial variability of hydraulic conductivity, were incorporated in the model. Both layered and randomly (log-normally) distributed physical and geochemical heterogeneities were considered. The upstream weighted multiple cell balance method was employed to numerically solve the governing equations of groundwater flow and virus transport. Model predictions show that the presence of subsurface layered geochemical and physical heterogeneity results in preferential flow paths and thus significantly affect virus mobility. Random distributions of physical and geochemical heterogeneity have also notable influence on the virus transport behavior. While the solution inactivation rate was found to significantly influence the virus transport behavior, surface inactivation under realistic field conditions has probably a negligible influence on the overall virus transport. It was further demonstrated that large virus release rates result in extended periods of virus breakthrough over significant distances downstream from the injection sites. This behavior suggests that simpler models that account for virus adsorption through a retardation factor may yield a misleading assessment of virus transport in "hydrogeologically sensitive" subsurface environments.  相似文献   
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ABSTRACT

Airborne measurements were made of gaseous and particulate species in the plume of a large coal-fired power plant after flue gas desulfurization (FGD) controls were installed. These measurements were compared with measurements made before the controls were installed. The light scattering and number and volume distributions of plume excess particles were determined by nephelometry and optical particle counting techniques. The plume impact based on optical techniques was much lower than that observed in earlier measurements. Indeed, plume excess volumes as a function of particle size were of the same magnitude as the variability of the background volume distribution. In situ excess plume scattering actually decreased with distance from the source, in contrast to pre-FGD conditions. The upper limit for the dry rate of SO2-to-SO4 2- conversion was estimated from plume excess volume measurements to be about 4% lir1. This is slightly greater than the upper limit, 3.5% lir1, estimated by earlier researchers, but the same as that estimated using the present technique with the earlier data. The cross-plume profile of volume suggests SO2-to-SO4 2- conversion is highest at the plume edges. The greatest benefit of SO2 reduction on plume excess volume and visibility appears to occur far downwind of the source.  相似文献   
9.
ABSTRACT

The rate of conversion of SO2 to SO4 2- was re-estimated from measurements made in the plume of the Cumberland power plant, located on the Cumberland River in north-central Tennessee, after installation of flue gas desulfurization (FGD) scrubbers for SO2 removal in 1994. The ratio of SO2 to NOy emissions into the plume has been reduced to ~0.1, compared with a prescrubber value of ~2. To determine whether the SO2 emissions reduction has correspondingly reduced plume-generated particulate SO4 2- production, we have compared the rates of conversion before and after scrubber installation. The prescrubber estimates were developed from measurements made during the Tennessee Plume Study conducted in the late 1970s. The post-scrubber estimates are based upon two series of research flights in the summers of 1998 and 1999. During two of these flights, the Cumberland plume did not mix with adjacent power plant plumes, enabling rate constants for conversion to be estimated from samples taken in the plume at three downwind distances. Dry deposition losses and the fact the fact that SO2 is no longer in large excess compared with SO4 2- have been taken into account, and an upper limit for the conversion rate constant was re-estimated based on plume excess aerosol volume. The estimated upper limit values are 0.069 hr-1 and 0.034 hr-1 for the 1998 and 1999 data, respectively. The 1999 rate is comparable with earlier values for nonscrubbed plumes, and although the 1998 upper limit value is higher than expected, these estimates do not provide strong evidence for deviation from a linear relationship between SO2 emissions and SO4 2- formation.  相似文献   
10.
The mechanism of bacterial-derived bioluminescence in the apogonid species Siphamia permutata and S. cephalotes presents some special structures, particularly the existence of two sites harboring bioluminescent bacteria, and not only one as previously described. One site, is the familiar disc-like, bacteria-harboring gland and paired ventral bioluminescent reflectors, typical for this genus, that merge and end at the level of the hypobranchial region. The second and more anteriorly situated site of luminescence features two sacs that originate in the gular region and protrude into the oral cavity, via the free space left by the laterally notched tongue. The apical parts of these luminous sacs harbor the bacteria whose light diffuses within the oral cavity. The tongue surface in the studied species is unique in character among the cardinal fishes, being criss-crossed anteriorly by protruding dermal ridges, rich in taste buds and mucus-producing cells. It would appear that at night when feeding, the luminous mouth cavity of these fish acts as a lure to attract the small prey, while the ridged structure of the tongues surface facilitates their collection and aggregation before they are swallowed.Communicated by O. Kinne, Oldendorf/Luhe  相似文献   
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