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We report the prenatal sonographic detection of a fetus with megalencephaly, polymicrogyria, postaxial polydactyly and hydrocephaly. Only 14 patients have been reported in the literature so far, all but one were diagnosed postnatally. The polymicrogyria in the frontoparietal lobe was confirmed by prenatal magnetic resonance imaging. Additionally, a hypoplastic thymus as seen in a 22q11 deletion was present. Although polymicrogyria along with pre-axial polydactyly has been described in 22q11 deletion, the diagnosis of Di George syndrome was ruled out. The etiology of megalencephaly, polymicrogyria, postaxial polydactyly and hydrocephaly has not been revealed yet. A dominant as well as recessive inheritance has been suggested. © 2013 John Wiley & Sons, Ltd.  相似文献   
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Energy consumption is related to local, regional and global impacts. Thus, by comparing different replacement scenarios of diesel vehicles with compressed natural gas, this article estimates pollutants and greenhouse gases emission in the city of São Paulo, Brazil. The calculation of fuel consumption is based on fleet characteristics, in terms of vehicle age, the average annual distance travelled by bus depending on the year and average fuel consumption. These values served as a basis to develop scenarios considering that a percentage of new vehicles that will be phased out and replaced with ones running on natural gas. Results show that the total avoided emissions can range from 579 thousand tons to 1.375 million tons of carbon dioxide (CO2) over 20 years, depending on the scenario. For particulate matter, accumulated avoided emissions vary from 251 thousand to 584 tons over 20 years. The replacement of diesel buses with natural gas-fuelled buses presents favourable results, in comparison with the tendency scenario for CO2 and particulate matter. Thus, a public policy for fuel replacement in largest cities, such as Sao Paulo, has an important global impact, especially when allowing the introduction of a renewable energy source, such as biogas. It will benefit from the natural gas previous infrastructure, which is largely available in Brazil. As recommendations, we explain the need to review the city of Sao Paulo Climate Change Law to allow the use of natural gas. Fuel replacement should be integrated with a public policy/public policies and operational strategies to promote citizens´ health as well as historical, cultural and heritage conservation for the city and its future generations.  相似文献   
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The need to collect data representative of overall urban pollution is all-important in order to monitor the population exposure. High spatial resolution monitoring using diffusive samplers allows studying of the urban pollutant distribution, thus enabling deeper investigation of their generation and diffusion mechanisms. Nevertheless, such a monitoring campaign has a certain cost. In this study we point out how to find the best compromise between the number of necessary measurements and the affordable costs for monitoring campaigns. We also describe an innovative method for the proper design of a fixed urban monitoring network by means of preliminary high spatial resolution campaigns using diffusive samplers. Four European capital cities (Dublin, Madrid, Paris and Rome) were monitored six times, each time for seven days. Benzene, toluene, ethylbenzene, xylenes (BTEX) and NO(2) concentrations were measured at 146 sites in Dublin, 293 in Madrid, 339 in Paris and 290 in Rome. Multiscale grids have been drawn which ranged in mesh size from 500 m to 2 km. The statistical processing of data produced a twofold result: the creation of isoconcentration maps with geostatistical procedures, and an algorithm aimed at locating the minimum number of sampling sites where the fixed monitoring stations should be placed. Average urban levels estimated on the basis of these selected sites differ by less than 8% from those calculated on the whole populations of the sampled points. The aim of this work is to investigate how far the resolution of a monitoring campaign of urban pollution by diffusive sampling can be reduced, thus making the monitoring less expensive in terms of human and financial resources, while preserving the same quality of the results that could be achieved with a higher resolution. We found that there is no significant loss of information when the resolution of the monitoring grid for BTEX is lowered to a mesh size of 1.85 km, that is a sampling site each 3.4 km(2), and that the minimum number of sampling sites to be used is N = 0.29 A, where A is the urban surface to be monitored (in km(2)). As the spatial distribution of NO(2) is less sensitive to the distance from the emission source than that of BTEX, this relationship could be retained as a valid lower limit for the mesh grid size also for NO(2) monitoring.  相似文献   
166.
The Gironde fluvial estuarine system is impacted by historic metal pollution (e.g. Cd, Zn, Hg) and oysters (Crassostrea gigas) from the estuary mouth have shown extremely high Cd concentrations for decades. Based on recent work (Chiffoleau et al., 2005) revealing anomalously high Ag concentrations (up to 65 mg kg−1; dry weight) in Gironde oysters, we compared long-term (∼1955-2001) records of Ag and Cd concentrations in reservoir sediment with the respective concentrations in oysters collected between 1979 and 2010 to identify the origin and historical trend of the recently discovered Ag anomaly. Sediment cores from two reservoirs upstream and downstream from the main metal pollution source provided information on (i) geochemical background (upstream; Ag: ∼0.3 mg kg−1; Cd: ∼0.8 mg kg−1) and (ii) historical trends in Ag and Cd pollution. The results showed parallel concentration-depth profiles of Ag and Cd supporting a common source and transport. Decreasing concentrations since 1986 (Cd: from 300 to 11 mg kg−1; Ag: from 6.7 to 0.43 mg kg−1) reflected the termination of Zn ore treatment in the Decazeville basin followed by remediation actions. Accordingly, Cd concentrations in oysters decreased after 1988 (from 109 to 26 mg kg−1, dry weight (dw)), while Ag bioaccumulation increased from 38 up to 116 mg kg−1, dw after 1993. Based on the Cd/Ag ratio (Cd/Ag ∼ 2) in oysters sampled before the termination of zinc ore treatment (1981-1985) and assuming that nearly all Cd in oysters originated from the metal point source, we estimated the respective contribution of Ag from this source to Ag concentrations in oysters. The evolution over the past 30 years clearly suggested that the recent, unexplained Ag concentrations in oysters are due to increasing contributions (>70% after 1999) by other sources, such as photography, electronics and emerging Ag applications/materials.  相似文献   
167.
Very few hydrological models commonly used in watershed management are appropriate for simulating the saturation excess runoff. The Soil Moisture Routing model (SMR) was developed specifically to predict saturation excess runoff from variable source areas, especially for areas where shallow interflow controls saturation. A recent version of SMR was applied to two rural catchments in the Catskill Mountains to evaluate its ability to simulate the hydrology of these systems. Only readily available meteorological, topographical, and landuse information from published literature and governmental agencies was used. Measured and predicted streamflows showed relatively good agreement; the average Nash–Sutcliffe efficiency for the two watersheds were R 2=72% and R 2=63%. Distributed soil moisture contents and the locations of hydrologically sensitive areas were also predicted well.  相似文献   
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Behavioral ecologists have often assumed that dispersal is costly mainly because of unfamiliarity with traversed habitats during dispersal and energy costs of the movement per se; thus, dispersers that have successfully settled should experience survival rates comparable to those of philopatric individuals. In this paper, we tested that hypothesis using 152 radio-collared European hares in a harvested population. We developed a multi-event capture recapture model, combining telemetry data and recoveries and separately modeling the foray probability, the settlement probability, and the permanent dispersal probability. The parameterization introduced here raises the possibility of separately testing effects on survival and dispersal probabilities at each stage of dispersal (departure, transience, and settlement). In accordance with our expectations, we reveal that dispersers incur higher mortality risks during transience and the early settlement period than philopatric individuals or settled dispersers. We also found that dispersers suffer from higher risks of being shot. Those results illustrate that unfamiliarity with the habitat during transience makes dispersal costly and that settled dispersers may enjoy survival rates comparable to those of philopatric individuals. Surprisingly, we also found that individuals have a higher probability of foraying during the hunting season. We suggest that hunting and related disturbances increase dispersal costs both by increasing mortality risk during transience and (perhaps) by increasing movement rates. We emphasize the need to take human pressures into account as factors that may drive the demographics of movements in populations.  相似文献   
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