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1.
Exencephaly was diagnosed at 17 weeks in a 27-year-old primigravida with abnormalities of the hands and a family history suggestive of autosomal dominant brachydactyly and clinodactyly. In this family there was also a history of ‘anencephaly’. To our knowledge, this is the first report on the association of exencephaly and autosomal dominant brachydactyly. As the relationship between hand and cranial anomalies is well established, we suggest that this association in our case could be due to a defect in the same gene.  相似文献   
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The biodegradation of aliphatic and aromatic hydrocarbons by natural soil microflora and seven fungi species, including imperfect strains and higher level lignolitic species, is compared in a 90-day laboratory experiment using a natural, not-fertilized soil contaminated with 10% crude oil. The natural microbial soil assemblage isolated from an urban forest area was unable to significantly degrade crude oil, whereas pure fungi cultures effectively reduced the residues by 26-35% in 90 days. Normal alkanes were almost completely degraded in the first 15 days, whereas aromatic compounds (phenanthrene and methylphenanthrenes) exhibited slower kinetics. Aspergillus terreus and Fusarium solani, isolated from oil-polluted areas, produced the more efficient attack of aliphatic and aromatic hydrocarbons, respectively. Overall, imperfect fungi isolated from polluted soils showed a somewhat higher efficiency, but the performance of unadapted, indigenous, lignolitic fungi was comparable, and all three species, Pleurotus ostreatus, Trametes villosus and Coriolopsis rigida, effectively degraded aliphatic and aromatic components. The simultaneous, multivariate analysis of 22 parameters allowed the elucidation of a clear reactivity trend of the oil components during biodegradation: lower molecular weight n-alkanes > phenanthrene > 3-2-methylphenanthrenes > intermediate chain length n-alkanes > longer chain length n-alkanes > isoprenoids approximately 9-1-methylphenanthrenes. Irrespective of the individual degrading capacities, all fungi species tested seem to follow this decomposition sequence.  相似文献   
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This paper explores linkages between food security and crisis in different contexts, outlining the policy and institutional conditions needed to manage food security during a crisis and to rebuild the resilience of food systems in periods of relative peace. The paper reviews experiences over the past decade of countries in protracted crisis and draws lessons for national and international policy. It assesses the different alternatives on offer in fragile countries to address, for example, the disruption of institutional mechanisms and the decreasing level of support offered by international donors with respect to longer-term expectations. It proposes a Twin Track Approach to enhance food security resilience through specific policies for protracted crises that link immediate hunger relief interventions with a long-term strategy for sustainable growth. Finally, the article analyses policy options and the implications for both short- and longer-term responses vis-à-vis the three dimensions of food security: availability; access; and stability.  相似文献   
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Land degradation is a process negatively affecting environmental sustainability and requires permanent monitoring for understanding its nonlinear trajectories of change over time and space. Environmental sustainability is linked to a theoretical definition of dynamic balance among various components contributing to the ecosystem quality and functioning. The aim of this study is to develop a diachronic analysis (1960–2010) of the equilibrium/disequilibrium condition of key environmental factors (climate, soil, vegetation, land-use) influencing the vulnerability of land to degradation in a Mediterranean country experiencing processes of desertification at the local scale. Three indicators of components’ balance have been proposed and tested for spatial and temporal coherence. Land classified at high vulnerability and low component’s balance has been identified as a possible target for mitigation strategies against desertification; the surface area of this class increased rapidly during 1960–2010 and concentrated in high-intensity agricultural lowlands of northern Italy.  相似文献   
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The temporal variability and bioaccumulation dynamics of C12–25 n-alkanes, isoprenoids and unresolved aliphatic hydrocarbons (UCM) were studied in a detritivorous fish (Sábalo: Prochilodus lineatus) collected from 1999 to 2005 in the sewage impacted Buenos Aires coastal area. Fish muscles contain huge amounts of n-C12–25 (165 ± 93, 70 ± 48 or 280 ± 134 μg g−1, dry, fresh and lipid weight, respectively) and UCM (931 ± 560, 399 ± 288 and 1567 ± 802 μg g−1) reflecting the chronic bioaccumulation of fossil fuels from sewage particulates. On a temporal basis, lipid normalized aliphatic concentrations peaked by the end of 2001–2002 during the rainiest period over the last four decades (1750 vs. 1083 ± 4.6 mm in 1999, 2004 and 2005), reflecting an enhanced exposition due to massive anthropogenic fluxes from Metropolitan Buenos Aires in wet years. The hydrocarbon composition in fish muscles is enriched in n-C15–17 and isoprenoids relative to a fresh crude oil and settling particulates, with fresher signatures during the 2001–2002 maxima. Fish/settling material bioaccumulation factors (BAFs: 0.4–6.4 dry weight or 0.07–0.94 lipid-organic carbon) plotted against Kow showed a parabolic pattern maximizing at n-C14–18 and isoprenoids. The optimal bioaccumulation window corresponds to highly hydrophobic (log Kow: 7.2–9.9), intermediate-size C14–18 n-alkanes and C15–20 isoprenoids (MW: 198–282; length: 17.9 to 25.4 Å) with melting points ranging from −19.8 to 28 °C. The uptake efficiency is inversely correlated to melting points and increased from 75% for n-C25 to above 90% for n-C14–15 and isoprenoids.  相似文献   
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The temporal variability and bioaccumulation dynamics of individual PCBs were studied in a detritivorous fish (Sábalo: Prochilodus lineatus) collected from 1999 to 2005 in the polluted Buenos Aires coastal area. Fish muscles contain high concentrations of total PCBs (11+/-7.2, 4.6+/-3.4 or 19+/-13 microg g(-1), dry, fresh and lipid weight, respectively) reflecting chronic bioaccumulation from sewage-industrial particulates. On a temporal basis, lipid normalized PCBs concentrations peaked by the end of 2001-2002 coincident with the rainiest period over the last four decades and shortly after PCB prohibition in the country, reflecting massive discharges to the coastal ecosystem. PCB composition in fish muscles show a prevailing contribution of hexachlorobiphenyls (35+/-4.2%), followed by hepta (23+/-3.0%), penta (20+/-3.6%), tri-tetra (16+/-4.8%) and minor proportions of octa-decachlorobiphenyls (5.7+/-3.1%) similar to an Aroclor 1242-1254-1260 1:2:4 mixture. During 2001-2002 maxima fish showed an enrichment in tri-tetrachlorobiphenyls ( approximately 1242-1254-1260 1:1:1 mixture) denoting a fresher signature. Fish/settling material lipid-organic carbon accumulation factors (BSAFs: 2.4-46, average: 21+/-10) plotted against kow showed a parabolic trend (BSAFs=-0.38 log kow2 + 5.16 log kow -15.85; R2=0.46) maximizing at hexa, hepta and octachlorobiphenyl 203 with reduced bioaccumulation of a few hepta (170, 191) and most octa-decachlorobihenyls suggesting limited intestinal absorption.  相似文献   
8.
Under what conditions do critical events trigger large-scale public discussion and mobilisation, and can these lead to policy change? In a comparative study of nuclear energy policy after the Japanese Fukushima disaster in March 2011, a theory-development approach is adopted, mobilising data collected from national news agencies’ newswires, public surveys, legislation and parliamentary databases, and newspaper editorials in 12 established democracies between March 2011 and March 2013. The analysis suggests two main hypotheses that can guide future research: critical events are more likely to trigger policy change when intense (contentious) mobilisation from policy challengers aligns with the views of the general public, and is backed by major political allies; and critical events are more likely to trigger intense (contentious) mobilisation when policy challengers articulate their opposition around pre-existing policy debates on the issue and resort to pre-existing organisational and mobilisation resources.  相似文献   
9.
The Taranto basin is a shallow water marine system in the South of Italy characterized by the presence of a lagoon environment together with a semi-enclosed bay connected to the Ionian Sea. This marine system experienced over the last few decades strong biochemical pollution and environmental degradation, and it is considered a hotspot study site for economic, ecological and scientific reasons. The aim of this study was to examine, on an annual temporal scale and with high spatial resolution, the main hydrodynamical processes and transport scales of the system by means of a 3D finite element numerical model application, adopting the most realistic forcing available. The model allowed us to assess the role played by baroclinic terms in the basin circulation, describing its estuarine nature. In particular, the main features of water circulation, salinity and temperature distribution, water renewal time and bottom stress were investigated. Our results allowed us to equate this system dynamic to that of a weakly stratified estuary, identifying the main driving sources of this mechanism. The vertical stratification over the whole year was proved to be stable, leading to a dual circulation flowing out on the surface, mainly through Porta Napoli channel, and inflowing on the bottom mainly through Navigabile channel. This process was responsible also for the renewal time faster on the bottom of the Mar Piccolo basin than the surface. Due to the great importance of the Taranto basin for what concerns sediment pollution, also the effect of currents in terms of bottom stress was investigated, leading to the conclusion that only in the inlets area the values of bottom stress can be high enough to cause erosion.  相似文献   
10.
Major and trace element, PAH, and PCB concentrations were measured in surface sediments and particles from sediment traps collected in the First and Second Basin of the Mar Piccolo (Gulf of Taranto) in two periods (June–July and August–September, 2013). The aim of the study was to evaluate pollution degree, sediment transport and particle redistribution dynamic within the area. Results confirm the higher contamination of sediments from the First Basin observed by previous researches, particularly for Cu, Hg, Pb, total PAHs, and total PCBs. Advective transport from the First to the Second Basin appears to be the leading transfer mechanism of particles and adsorbed contaminants, as evidenced by measured fluxes and statistical analyses of contaminant concentrations in surficial sediments and particles from sediment traps. Long-range selective transports of PAHs and microbial anaerobic degradation processes for PCBs have been also observed. These results are limited to a restricted time window but are consistent with the presence of transport fluxes at the bottom of the water column. This mechanism deserves further investigation and monitoring activities, potentially being the main responsible of pollutant delivering to the less contaminated sectors of the Mar Piccolo.  相似文献   
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