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41.
We studied two children born to a myasthenic mother. The first child, a female, had multiple flexion contractures. She died 1 h after birth. In the second pregnancy, 3 years later, ultra-sonographic examination at 20 weeks showed decreased fetal movements and multiple flexion contractures. The pregnancy was interrupted. Eight other cases of congenital rnyasthenia with arthrogryposis are known; four of them are siblings. The recurrence risk may be as high as 100 per cent. Our second case demonstrates that prenatal diagnosis is possible early enough to allow termination of pregnancy.  相似文献   
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Real-time methods to monitor stable isotope ratios of CO_2 are needed to identify biogeochemical origins of CO_2 emissions from the soil–air interface. An isotope ratio infra-red spectrometer(IRIS) has been developed to measure CO_2 mixing ratio with δ~13C isotopic signature, in addition to mixing ratios of other greenhouse gases(CH_4, N2_O). The original aspects of the instrument as well as its precision and accuracy for the determination of the isotopic signature δ~13C of CO_2 are discussed. A first application to biodegradation of hydrocarbons is presented, tested on a hydrocarbon contaminated site under aerobic bio-treatment. CO_2 flux measurements using closed chamber method is combined with the determination of the isotopic signature δ~13C of the CO_2 emission to propose a non-intrusive method to monitor in situ biodegradation of hydrocarbons. In the contaminated area, high CO_2 emissions have been measured with an isotopic signature δ~13C suggesting that CO_2 comes from petroleum hydrocarbon biodegradation.This first field implementation shows that rapid and accurate measurement of isotopic signature of CO_2 emissions is particularly useful in assessing the contribution of contaminant degradation to the measured CO_2 efflux and is promising as a monitoring tool for aerobic bio-treatment.  相似文献   
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Recent large-scale studies have shown that biodiversity-rich regions also tend to be densely populated areas. The most obvious explanation is that biodiversity and human beings tend to match the distribution of energy availability, environmental stability and/or habitat heterogeneity. However, the species–people correlation can also be an artefact, as more populated regions could show more species because of a more thorough sampling. Few studies have tested this sampling bias hypothesis. Using a newly collated dataset, we studied whether Orthoptera species richness is related to human population size in Italy’s regions (average area 15,000 km2) and provinces (2,900 km2). As expected, the observed number of species increases significantly with increasing human population size for both grain sizes, although the proportion of variance explained is minimal at the provincial level. However, variations in observed Orthoptera species richness are primarily associated with the available number of records, which is in turn well correlated with human population size (at least at the regional level). Estimated Orthoptera species richness (Chao2 and Jackknife) also increases with human population size both for regions and provinces. Both for regions and provinces, this increase is not significant when controlling for variation in area and number of records. Our study confirms the hypothesis that broad-scale human population–biodiversity correlations can in some cases be artefactual. More systematic sampling of less studied taxa such as invertebrates is necessary to ascertain whether biogeographical patterns persist when sampling effort is kept constant or included in models.  相似文献   
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Abstract: In 1997, the Amazon Basin experienced an exceptionally severe El Niño drought. We assessed effects of this rare event on mortality rates of trees in intact rain forest based on data from permanent plots. Long-term (5- to 13-year) mortality rates averaged only 1.12% per year prior to the drought. During the drought year, annual mortality jumped to 1.91% but abruptly fell back to 1.23% in the year following El Niño. Trees dying during the drought did not differ significantly in size or species composition from those that died previously, and there was no detectable effect of soil texture on mortality rates. These results suggest that intact Amazonian rainforests are relatively resistant to severe El Niño events.  相似文献   
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Solvent toxicity to amphibian embryos and larvae   总被引:1,自引:0,他引:1  
Organic micropollutants are often damaging for aquatic organisms. Being usually hydrophobic compounds, they are often dissolved in an organic co-solvent which increases their solubility in water. The aim of this study was to study the toxicity of various solvents on embryos (protected or not by jelly coat) and on tadpoles of the common frog (Rana temporaria). Tested solvents were methanol (MeOH), methylene chloride (CH(2)Cl(2)), dimethyl sulfoxyde (DMSO), acetone (Ac) and ethanol (EtOH). Embryos exhibited higher mortality rates than tadpoles. Embryos with jelly were more sensitive to high concentration of solvents than embryos without jelly (except for acetone). According to these results, Ac, DMSO and CH(2)Cl(2) can be used as co-solvents in water to help the dissolution of micropollutants at concentration equal to or lower than 0.001 ml/l for frog embryos, and EtOH, Ac and CH(2)Cl(2) at concentration equal to or lower than 0.01 ml/l for Rana temporaria tadpoles.  相似文献   
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Two statistical methods for the analysis of data on the evolution of the chemical composition of cold snow (<0°C) in the field (Lac Laflamme, Quebec) were compared. The methods used on the data were regression analysis (One sample per sampling date over a long cold period) and ANOVA (replicate samples on a restricted number of sampling dates over shorter periods). The relative power of the tests to determine the detectable amplitude of chemical changes was derived from the theoretical power of the tests under comparable conditions of sampling (number of observations) and from the estimated error variances of the measured data.The results of the study on the evolution of sulfates (SO4) concentrations in discretely identified snow strate clearly showed that for six of the eight strata, significant losses of SO4 occurred in snow during cold periods. The relative amplitude of the significant losses varied between 1% per day and 4% per day depending on the initial concentrations in the snow and the prevailing meteorological conditions.The analysis of the data also demonstrated that for the same number of samples, the regression analysis is more efficient in detecting the chemical changes in snow than the alternative ANOVA method. The use of this information to plan sampling programs of cold snow under both field and laboratory conditions is discussed.  相似文献   
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