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Prenatal diagnosis in a pregnancy at risk for a juvenile B1 variant of GM2-gangliosidosis was carried out. The biochemical study of the cultured amniocytes and the affected fetal brain is reported. The results obtained show that the sulphated artificial substrate can be used in the diagnosis of B1 variant, but not the neutral one. The accumulation of GM2-ganglioside in the fetal brain of the B1 juvenile form and an infantile form of GM2-gangliosidosis (0 variant) was compared.  相似文献   
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Abstract

Organochlorine pesticides (OCPs) are widely used around the world as insecticides, herbicides, fungicides, nematicides, and rodenticides. Despite banned in Brazil, the usage remains occurring in many countries. The persistence and extreme mobility of OCPs contribute to the contamination of the environment and the human body. The OCPs bioaccumulation in adipose tissue triggers the excretion into human milk during breastfeeding. Hence, the present study determined eighteen OCPs residues in the breast milk of mothers from the Western Region of Bahia State, Brazil. Nine different residue species were found, including beta-Hexachlorocyclohexane (9.24?±?0.00?ng g?1 fat), delta- Hexachlorocyclohexane (22.15?±?10.48?ng g?1 fat), Heptachlor (58.08?±?74.13?ng g?1 fat), Aldrin (142.65?±?50.65?ng g?1 fat), Dieldrin (774.62?±?472.68?ng g?1 fat), Endosulfan I (408.44?±?245.51?ng g?1 fat), Dichloro-diphenyl-dichloro-ethylene (29.17?±?22.42?ng g?1 fat), Dichloro-diphenyl-trichloro-ethane (28.87?±?0.00?ng g?1 fat) and Methoxychlor (1699.67?±?797.43?ng g?1 fat). The Methoxychlor presence in all samples may reveal a recent exposure, while Dieldrin and Endosulfan I analyses can point to distant past exposure.  相似文献   
4.
Environmental Science and Pollution Research - The development of building materials using new types of raw materials is currently on demand by society and the industry. It is intended to reduce...  相似文献   
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The mechanism of flow turbulence,sediment supply conditions,and sediment transport patterns that affect the adsorption of cadmiumions onto sediment particles in natural waters are experimentally simulated and studied both in batch reactors and in a turbulencesimulation tank.By changing the agitation conditions,the sediment transport in batch reactors can be categorized into bottom sediment-dominated sediment and suspended sediment-dominated sediment.It is found that the adsorption rate of bottom sediment is much lessthan that of suspended sediment,but the sediment transport pattern does not affect the final(equilibrium)concentration of dissolvedcadmium.This result indicates that the parameters of an adsorption isotherm are the same regardless of the sediment transport pattern.Inthe turbulence simulation tank,the turbulence is generated by harmonic grid-stirred motions,and the turbulence intensity is quantifiedin terms of eddy diffusivity,which is equal to 9.84F(F is the harmonic vibration frequency)and is comparable to natural surfacewater conditions.When the turbulence intensity of flow is low and sediment particles stay as bottom sediment,the adsorption rateis significantly low,and the adsorption quantity compared with that of suspended sediment is negligible in the 6 h duration of theexperiment.This result greatly favors the simplification of the numerical modeling of heavy metal pollutant transformation in naturalrivers.When the turbulence intensity is high but bottom sediment persists,the rate and extent of descent of the dissolved cadmiumconcentration in the tank noticeably increase,and the time that is required to reach adsorption equilibrium also increases considerablydue to the continuous exchange that occurs between the suspended sediment and the bottom sediment.A comparison of the results ofthe experiments in the batch reactor and those in the turbulence simulation tank reveals that the adsorption ability of the sediment,andin particular the adsorption rate,is greatly over-estimated in the batch reactor.  相似文献   
6.
The several established criteria to define a hyperaccumulator plant were applied to a rare and endangered species, Plantago almogravensis, and to the 3rd most abundant element in the earth crust, Al. Using the most common criteria, P. almogravensis undoubtedly is an Al hyperaccumulator plant. If the recent proposed requirements were considered, most of them matching those for a plant to be used in phytoextraction, it can only be considered an unusual accumulator of Al. A discussion is made concerning the several criteria of a hyperaccumulator plant in order to include rare and endemic ones and abundant elements. In ecological terms, the enrichment in Al and Fe observed may account for the differences in the vegetation pattern. Due to the rarity and endangered nature of this plant, the contribution of this work is also relevant for the ecological understanding and the development of conservation options of this endemic species.  相似文献   
7.
Current demand analysis methods do not formally cover the case of chronic deficits in quantity or quality of water and sanitation services. These services include drinking water supply (DWS), wastewater and sewage treatment (WST), and municipal solid waste management (MSW). Formal analysis of this case would, at minimum, define the deficit state and evaluate appropriate options for reducing it. This paper proposes for a formal analytical model for municipal sanitation systems (MSS) that operate with deficits in at least one of the constituent services of DWS, WST, or MSW. The model introduces definitions and notation for describing the deficit state for conducting demand analysis on municipal sanitation systems. This model of demand analysis for systems with chronic deficits will hereinafter be referred to as deficit analysis. A case study for Bacoor, Philippines is presented as an example.  相似文献   
8.
Despite the often mentioned environmental benefits associated with transition from fossil fuels to renewable energy sources, their use for electricity production has non-negligible negative environmental impacts. The most commonly mentioned in surveys concern different types of landscape impacts, impacts on the fauna and flora, and noise. These impacts differ by size and location of plants, and by source of energy, rendering the policy decision complex. In addition, there are other welfare issues to take into consideration, as positive and negative environmental impacts are not evenly distributed among population groups. This paper proposes to compare the welfare impacts of renewable energy sources controlling for the type of renewable as well as the specific environmental impact by source. To this end, two discrete-choice experiments are designed and applied to a national sample of the Portuguese population. In one case, only individual negative impacts of renewables are used, and in another case, the negative impacts interact with a specific source. Results show the robustness of discrete-choice experiments as a method to estimate the welfare change induced by the impacts of renewable energy sources. Overall, respondents are willing to pay to reduce the environmental impacts, thus making compensation for local impacts feasible. Moreover, the estimations reveal that respondents are significantly sensitive to the detrimental environmental effects of specific renewable energy sources, being willing to pay more to use these sources of energy relative to others.  相似文献   
9.
建立了非稳态过滤下的固定床颗粒层除尘效率模型.该模型尽可能地包含了影响固定床颗粒层除尘的参数,反映了沉积粉尘对除尘效率的影响;研究了以粒状脱硫剂为滤料的固定床在不同颗粒层厚度、空床气速、粒径下的除尘效率.结果表明,在颗粒层厚度为400~800 cm、空床气速≤0.4 m/s的条件下,颗粒层的除尘效率可达90%以上.  相似文献   
10.
Remodelling works are frequent in harbour areas, given that they must adapt to rapidly changing market requirements. Depending on their nature (construction, demolition, etc.), these works may be carried out during long periods of time and thus exert a significant impact on the air quality at the harbours and their surroundings. The air quality at the Valencia harbour was studied during an enlargement project. Four sampling stations aimed to cover the major dust-emitting activities in the harbour. In addition, a sampling campaign was carried out to collect dust materials at their emission sources (e.g., loading and unloading of clinker and petroleum coke, as well as the enlargement works). Differences obtained between PM levels at the monitoring sites were correlated with the distance between sampling stations and enlargement works and/or harbour operations, as well as with the preferential wind direction. In all cases, the days with the highest PM_10 levels were linked to wind directions coinciding with the location of the enlargement works or the clinker and petroleum coke stocks. Classic source apportionment methodologies (PCA and CMB) were applied to the data, but their interpretation was complex due to the similar chemical signatures of PM originating from direct and fugitive emissions from stocked materials (e.g., clinker), and the enlargement works. To overcome this difficulty, a novel non-statistical approach was used to obtain quantitative estimations of the contributions from sources (petroleum coke and clinker), based on the correlation between specific PM components (e.g., carbonaceous species) and source tracers (e.g., V). Finally, a qualitative test using phenolphthalein was devised to identify the presence of clinker on the filters, which provided positive results. This novel tracer approach is thus recommended for harbour authorities.  相似文献   
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