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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 respiratory physiology of summer diapausing eggs of the neustonic copepodAnomalocera patersoni, maintained under constant temperature (13 °C) and light (12 h light:12 h dark) conditions, was characterized by a bell-shaped curve, with low O2 uptake levels at the beginning of dormancy. This was followed by a steady rise in O2 consumption with maximum levels of 0.002 l O2 embryo–1 h–1 70 d after spawning. A slow diminution in O2 uptake then occurred until Day 150 when minimum values of 0.0003 l O2 embryo–1 h–1 were recorded, coinciding with the hatching of the first embryos. Embryos continued to hatch asynchronously up to 360 d from the moment of egg laying. When eggs were subjected to 20 °C, the respiratory activity was almost three times higher than at 13 °C, even though both respiratory curves were similar. The elevated metabolism in eggs kept at 20 °C led to death of the embryos possibly due to a total depletion of metabolic reserves. ATP content also differed at the two temperatures. Diapause eggs kept at 20 °C showed no rapid rise in ATP content as opposed to those kept at 13 °C. The results of temperature shock experiments, in which eggs were first kept at winter temperatures for several weeks, after which the temperature was raised to 20 °C for another number of weeks prior to a second period of chilling at 13 °C, showed that as long as embryos were kept at 20 °C no hatching occurred. By contrast, hatching was observed after 10 d following the resumption of winter temperatures, suggesting that low environmental temperatures are an essential prerequisite for hatching of these eggs. The type of diapause inA. patersoni differs considerably from the one described in insects and in another neustonic copepod,Pontella mediterrana. In this case, there is a U-shaped respiratory curve with greatest O2 consumption prior to the onset or upon breaking of diapause. Differences in the two types of diapause seem to involve not only differences in O2 consumption levels but also in the sequence of metabolic changes with time and the metabolic requirements during sommer and winter dormancy.  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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Recent studies suggest that many organisms actively colonize the subterranean environment to avoid climatic stress, exploit new ecological opportunities and reduce competition and predation. Terrestrial salamanders are known to colonize the more stable subterranean habitats mainly to escape external climatic extremes, while the role of predation avoidance remains untested. To better understand the importance of predation, we used clay models of the cave salamander Speleomantes strinatii to compare the predation occurring in woodland and subterranean habitats. Models were positioned in three forests and in three caves in NW Italy. One-hundred eighty-four models were retrieved from the field and 59 (32%) were attacked by predators. Models were attacked on their head more often than expected by chance and, therefore, were perceived by predators as real prey items. In the woodlands, clay models showed a four-time higher probability of being attacked in comparison to caves, suggesting a different level of potential predation risk in these surface habitats. These findings are one of the first experimental evidences that, in terrestrial ecosystems, predation avoidance may contribute to the salamander underground colonization process.  相似文献   
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Many small terrestrial vertebrates exhibit limited spatial movement and are considerably exposed to changes in local environmental variables. Among such vertebrates, amphibians at present experience a dramatic decline due to their limited resilience to environmental change. Since the local survival and abundance of amphibians is intrinsically related to the availability of shelters, conservation plans need to take microhabitat requirements into account. In order to gain insight into the terrestrial ecology of the spectacled salamander Salamandrina perspicillata and to identify appropriate forest management strategies, we investigated the salamander’s seasonal variability in habitat use of trees as shelters in relation to tree features (size, buttresses, basal holes) and environmental variables in a beech forest in Italy. We used the occupancy approach to assess tree suitability on a non-conventional spatial scale. Our approach provides fine-grained parameters of microhabitat suitability and elucidates many aspects of the salamander’s terrestrial ecology. Occupancy changed with the annual life cycle and was higher in autumn than in spring, when females were found closer to the stream in the study area. Salamanders showed a seasonal pattern regarding the trees they occupied and a clear preference for trees with a larger diameter and more burrows. With respect to forest management, we suggest maintaining a suitable number of trees with a trunk diameter exceeding 30 cm. A practice of selective logging along the banks of streams could help maintain an adequate quantity of the appropriate microhabitat. Furthermore, in areas with a presence of salamanders, a good forest management plan requires leaving an adequate buffer zone around streams, which should be wider in autumn than in spring.  相似文献   
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