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71.
Prenatal diagnosis of chronic granulomatous disease (CGD) was performed in four male high risk fetuses. The male sex was previously determined by an amniotic cell karyotype. Three kinds of test were performed on fetal blood obtained by umbilical venous puncture under fetoscopy at the 20th gestational week: nitroblue tetrazolium reduction (NBT) cytochemical test with phorbol myristate acetate (PMA) as activator; luminol enhanced chemiluminescence with activation by serum opsonized zymosan (STZ) or PMA; superoxide anion (0) production by measurement of the superoxide dismutase inhibitable reduction of cytochrome c with PMA as activator. Results were compared to those obtained in six fetuses investigated for other inherited diseases. In one case, absence of granulocyte defects was confirmed at birth. In three other cases, the tests showed deficient metabolic oxidative granulocytes. The pregnancy was terminated and the CGD diagnosis was confirmed on the products of abortion. The use of three different techniques performed on whole blood for CGD prenatal diagnosis is recommended instead of a single isolated test to ensure a higher confidence in the diagnosis.  相似文献   
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ABSTRACT: Many urban and suburban communities in the Midwest are seeking to establish sustainable, morphologically and hydraulically varied, yet dynamically stable fluvial systems that are capable of supporting healthy, biologically diverse aquatic ecosystems — a process known as stream naturalization. This paper describes an integrated research program that seeks to develop a scientific and technological framework to support two stream naturalization projects near Chicago, Illinois. The research program integrates theory and methods in fluvial geomorphology, aquatic ecology, hydraulic engineering and social theory. Both the conceptual and the practical challenges of that integration are discussed. Scientific and technical support emphasize the development of predictive tools to evaluate the performance of possible naturalization designs at scales most appropriate to community based projects. Social analysis focuses on place based evaluations of how communities formulate an environmental vision and then, through decision making, translate this vision into specific stream naturalization strategies. Integration of scientific and technical with social components occurs in the context of community based decision making as the predictive tools are employed by project scientists to help local communities translate their environmental visions into concrete environmental designs. Social analysis of this decision making process reveals how the interplay between the community's vision of what they want the watershed to become, and the scientific perspective on what the watershed can become to achieve the community's environmental goals, leads to the implementation of specific stream naturalization practices.  相似文献   
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Color is one of the most important features in any textile material. Due to its competitive price, most of the colorants currently used for textile dyeing are synthetic, originated from nonrenewable sources, and highly pollutant. There is an increasing interest for natural processes to dye fabrics. When new textile dyeing technologies are developed, evaluating the quality of these techniques involves measuring the resulting color homogeneity using digital images. The presence of a texture effect, caused by the interlacing of warp and weft yarns, as well as small displacement of the fabric, creates a sophisticated dependence structure in pixels coloring. A random effects model is employed in order to separate the signal from the dyeing effect (fixed effect described by smooth functions) and warp and weft texture effect (Gaussian mixture driven by a hidden Markov random field), allowing an evaluation of color homogeneity in dyed textiles regardless of the effect of the texture.  相似文献   
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This study presents the experimental and modeling results of CO2 injection and transport in the vadose zone performed in PISCO2 facilities at the ES.CO2 center in Ponferrada (North Spain). During 46 days of experiments, 62.10 kg of CO2 were injected through 16 micro‐injectors in a 35 m3 experimental unit filled with sandy material. Monitoring and mapping of surface CO2 flux were performed periodically to assess the evolution of CO2 migration through the soil and to the atmosphere. Numerical simulations were run using TOUGH2 code with EOS7CA research module considering two phases (gas and liquid) and three components (H2O, CO2, air). Two layers (sand, gravel) and atmosphere boundary were implemented taking into account heterogeneous soils, homogeneous soil, rainfall, temperature, and liquid saturation to allow a better understanding of CO2 behavior in the vadose zone. This combined experimental and modeling approach shows that CO2 leakage in the vadose zone quickly comes out through preferential migration pathways and spots with the ranges of fluxes in the ground/surface interface from 2.5 to 600 g·m−2·day−1. This gas channeling is mainly related to soil compaction and climatic perturbation. This has significant implications for design‐adapted detection and monitoring strategies of early leakage in commercial CO2 storage. © 2015 Society of Chemical Industry and John Wiley & Sons, Ltd  相似文献   
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No-take reserves are sometimes implemented for sustainable population harvesting because they offer opportunities for animals to spatially avoid harvesters, whereas harvesters can benefit in return from the reserve spillover. Here, we used the framework of predator-prey spatial games to understand how protected areas shape spatial interactions between harvesters and target species and determine animal mortality. In these spatial games, the "predator" searches for "prey" and matches their habitat use, unless it meets spatial constraints offering the opportunity for prey to avoid the mortality source. However, such prey refuges could attract predators in the surroundings, which questions the potential benefits for prey. We located, in the Geneva Basin (France), hunting dogs and wild boar Sus scrofa L. during hunting seasons with global positioning systems and very-high-frequency collars. We quantified how the proximity of the reserve shaped the matching between both habitat uses using multivariate analyses and linked these patterns to animals' mortality with a Cox regression analysis. Results showed that habitat uses by both protagonists disassociated only when hunters were spatially constrained by the reserve. In response, hunters increased hunting efforts near the reserve boundary, which induced a higher risk exposure for animals settled over the reserve. The mortality of adult wild boar decreased near the reserve as the mismatch between both habitat uses increased. However the opposite pattern was determined for younger individuals that suffered from the high level of hunting close to the reserve. The predator-prey analogy was an accurate prediction of how the protected area modified spatial relationships between harvesters and target species. Prey-searching strategies adopted by hunters around reserves strongly impacted animal mortality and the efficiency of the protected area for this harvested species. Increasing reserve sizes and/or implementing buffer areas with harvesting limitations can dampen this edge effect and helps harvesters to benefit durably from source populations of reserves. Predator-prey spatial games therefore provide a powerful theoretical background for understanding wildlife-harvester spatial interactions and developing substantial application for sustainable harvesting.  相似文献   
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Dairy cows are responsible for a fair amount of gas emissions in the atmosphere (mainly methane, ammonia, and carbon dioxide), as well as waste outputs. Therefore, identifying high‐fertility breeding cows and increasing fertility rates can diminish pollution and help minimize the effect of global warming and improve the environmental impact of the farming system. As a step to achieve this goal, changes in the lipid composition of the bovine uterus exposed to greater (LF‐LCL group) or lower (SF‐SCL group) concentrations of progesterone during postovulation were investigated by matrix‐assisted laser desorption ionization mass spectrometry. Two measurements were made for each cow, and after preprocessing the data, the measurements available to analysis consist of relative intensities at significant 76 mass‐to‐charge ratio (m/z) values identifying specific ions in the spectra. Due to the small sample size, seven cows in the LF‐LCL group and 10 cows in the SF‐SCL group, the usual methods could not discriminate between groups. A model‐based approach was therefore proposed, and due to the discrete nature of the data, a truncated mixture of bivariate beta distributions was fitted to the data using an expectation–maximization algorithm. However, unlike the usual approach for mixture density estimation problems, to each 76 m/z value, we assign an unobserved label shared by all cows in the same group. The role of these labels is similar to the frailty effect in survival models in which all cows in a given group would share some random effect due to group effect. These labels will be used to identify m/z values, which could potentially account for different fertility rates.  相似文献   
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