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91.
Manifestations of Fanconi Anemia Complementation Group C (FA-C) include multiple major congenital malformations, hypoplastic radius, absent thumb, growth retardation, elfin-like facial features, microphthalmia, microcephaly, café-au-lait spots, early onset of hematologic disease and poor survival (Auerbach, 1997). We describe two cases in which second-trimester sonographic findings led to parental carrier testing for FA-C and subsequent prenatal diagnosis of affected fetuses. Copyright © 2005 John Wiley & Sons, Ltd.  相似文献   
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The high seas provide a variety of ecosystem services that benefit society. There have, however, been few attempts to quantify the human welfare impacts of changes to the delivery of these benefits. We assessed the values of several key ecosystem service benefits derived from protecting ecosystems in the high seas of the Flemish Cap through choice experiments conducted in Canada, Norway, and Scotland. Rather than solely eliciting public willingness to pay, we also explored the determinants of variance in the estimates of willingness to pay. We aimed to determine how much respondents were willing to pay for high-seas ecosystems conservation, which factors influence individuals’ willingness to pay, and whether individuals in Canada had a higher willingness to pay relative to those living in Norway and Scotland. This latter point captures distance-decay effects. On average, the public placed positive value on conserving high-seas ecosystems and on developing economic activities related to the exploitation and exploration of marine resources, despite a lack of awareness and familiarity with these environments. Distance-decay effects on willingness to pay were not clear. Scots had the highest willingness to pay and the Norwegians the lowest willingness to pay for all attributes, with the only exception being willingness to pay for a large increase in new jobs, in which case Canadians’ willingness to pay was higher than Scots’. The public's willingness to pay was influenced by sociodemographic characteristics and their perceptions of high-seas ecosystems. Our results provide evidence of the impacts of high-seas governance on human welfare and that improved governance could increase the value people place on high-seas ecosystems and the services they produce.  相似文献   
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Becoming proficient at navigation in urban environments is something that we all aspire to. Here we asked whether being an expert at wayfinding in one environment has any effect on learning new spatial layouts. Licensed London taxi drivers are among the most proficient urban navigators, training for many years to find their way around a complex and irregularly-laid out city. We first tested how well they could learn the layout of an unfamiliar town compared with a group of non-taxi drivers. Second, we investigated how effectively taxi drivers could integrate a new district into their existing spatial representation of London. We found that taxi drivers were significantly better than control participants at executing routes through the new town, and representing it at a map-like survey level. However, the benefits of navigational expertise were not universal. Compared with their performance in the new town, taxi drivers were significantly poorer at learning the layout of a new area that had to be integrated with their existing knowledge of London. We consider reasons for this picture of facilitation and limitation, in particular drawing parallels with how knowledge acquisition occurs in the context of expertise in general.  相似文献   
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The purpose of technology transfer under the UNFCCC Article 4.5 is to “…promote, facilitate, and finance as appropriate the transfer of, or access to, environmentally sound technologies and know how to other Parties particularly Developing Country parties to enable them to implement the provisions of the Convention.” The key challenge in this respect is that low-carbon sustainable technologies need to be adopted both by developed as well as developing countries. However, this paper focuses on the process of technology transfer to developing countries to allow them to move quickly to environmentally sound and sustainable practices, institutions and technologies. In the above framework, this paper reviews key aspects of technology transfer from a range of perspectives in the literature and discusses insights from this literature for the transfer and innovation process needed to reduce global vulnerability to climate change in the context of current international activities based on the research undertaken by the EU sponsored ENTTRANS project.  相似文献   
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There is little to no toxicity information regarding thousands of chemicals to which people are exposed daily. In fact, of the 84,000 chemicals listed in the United States Toxic Substances Control Act Inventory, there is limited information available on their effects on neural development (Betts, 2010 and US EPA, 2015). The number of chemicals tested remains low due to the high cost of conducting multi-generational animal studies and the lack of alternative testing methods.  相似文献   
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This study is a part of an ongoing investigation of the types and locations of emission sources that contribute fine particulate air contaminants to Underhill, VT. The air quality monitoring data used for this study are from the Interagency Monitoring of Protected Visual Environments network for the period of 2001-2003 for the Underhill site. The main source-receptor modeling techniques used are the positive matrix factorization (PMF) and potential source contribution function (PSCF). This new study is intended as a comparison to a previous study of the 1988-1995 Underhill data that successfully revealed a total of 11 types of emission sources with significant contributions to this rural site. This new study has identified a total of nine sources: nitrate-rich secondary aerosol, wood smoke, East Coast oil combustion, automobile emission, metal working, soil/dust, sulfur-rich aerosol type I, sulfur-rich aerosol type II, and sea salt/road salt. Furthermore, the mass contributions from the PMF identified sources that correspond with sampling days with either good or poor visibility were analyzed to seek possible correlations. It has been shown that sulfur-rich aerosol type I, nitrate aerosol, and automobile emission are the most important contributors to visibility degradation. Soil/dust and sea salt/road salt also have an added effect.  相似文献   
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