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Thierry Rousseau Nicole Laurent Christel Thauvin-Robinet Stéphanie Lionnais Christine Durand Laurence Faivre Paul Sagot 《黑龙江环境通报》2002,22(8):692-696
Fraser syndrome (MIM 219000) is a rare disorder of autosomal recessive inheritance, characterized by the association of cryptophthalmos, syndactyly and genital abnormalities. Here we report on two cases of Fraser syndrome (cryptophthalmos syndrome) in a non-consanguineous couple, with variable expression in echographic, clinical and autopsy findings. Furthermore, we highlight the difficulties in prenatal diagnosis of Fraser syndrome. Copyright © 2002 John Wiley & Sons, Ltd. 相似文献
3.
Stéphanie Couvreur-Lionnais Thierry Rousseau Nicole Laurent Christel Thauvin-Robinet Eve Senet-Lacombe Anne Lise Delezoïde Francine Mugneret Christine Durand Laurence Faivre Paul Sagot 《黑龙江环境通报》2005,25(2):172-175
Juberg–Hayward syndrome is a rare autosomal recessive syndrome characterised by the association of growth retardation, microcephaly, cleft lip and palate, and thumb and radial ray abnormalities. To date, no prenatal cases have been reported. Here, we report on the first prenatal case of Juberg–Hayward syndrome. The diagnosis was established following fetopathological study. Besides the cardinal features of the syndrome, this prenatal case was remarkable for the severity of the short arm malformation and by the finding of big toe agenesis and cerebral abnormalities including hydrocephalus, agenesis of corpus callosum, and cerebellar hypoplasia. We conclude that the diagnosis of Juberg–Hayward syndrome can be discussed prenatally following ultrasound diagnosis of the association of intrauterine growth restriction, microcephaly, thumb/radial anomalies, and cleft lip/palate. Copyright © 2005 John Wiley & Sons, Ltd. 相似文献
4.
The involvement of buses in accidents usually is assessed implicitly on the basis of the direct involvement of the bus in the collision or in injury production. This paper deals with the scope and forms of indirect involvement of buses (as a sight obstruction, for example). Accidents were selected by identifying the presence of the term ‘bus’ or synonyms in the text parts of complete police reports (testimonies, statements by the persons involved, etc.) available in electronic form, then analysed in detail. Direct or indirect involvement of a bus is found in 3.6% of traffic injury accidents reported by the police in the community studied (direct involvement: 1.4%; indirect involvement: 2.2%). The different forms of indirect involvement are then described, and some possibilities of preventive measures are discussed. 相似文献
5.
Céline Jézéquel Pablo A. Tedesco William Darwall Murilo S. Dias Renata G. Frederico Max Hidalgo Bernard Hugueny Javier Maldonado-Ocampo Koen Martens Hernan Ortega Gislene Torrente-Vilara Jansen Zuanon Thierry Oberdorff 《Conservation biology》2020,34(4):956-965
Conserving freshwater habitats and their biodiversity in the Amazon Basin is a growing challenge in the face of rapid anthropogenic changes. We used the most comprehensive fish-occurrence database available (2355 valid species; 21,248 sampling points) and 3 ecological criteria (irreplaceability, representativeness, and vulnerability) to identify biodiversity hotspots based on 6 conservation templates (3 proactive, 1 reactive, 1 representative, and 1 balanced) to provide a set of alternative planning solutions for freshwater fish protection in the Amazon Basin. We identified empirically for each template the 17% of sub-basins that should be conserved and performed a prioritization analysis by identifying current and future (2050) threats (i.e., degree of deforestation and habitat fragmentation by dams). Two of our 3 proactive templates had around 65% of their surface covered by protected areas; high levels of irreplaceability (60% of endemics) and representativeness (71% of the Amazonian fish fauna); and low current and future vulnerability. These 2 templates, then, seemed more robust for conservation prioritization. The future of the selected sub-basins in these 2 proactive templates is not immediately threatened by human activities, and these sub-basins host the largest part of Amazonian biodiversity. They could easily be conserved if no additional threats occur between now and 2050. 相似文献
6.
Grasslands were once widespread and species-rich ecosystems. They have drastically decreased throughout the world, largely due to changes in land-uses. Remnant grasslands are often highly degraded and disconnected and require active conservation. In order for grasslands to be maintained worldwide, protected areas need to be created. While adequately creating and managing protected areas has proven difficult, this can be improved by following a three-point guideline: (1) consider many ecological groups (birds, insects, plants); (2) use conservation biology knowledge; (3) seek agreements with concerned parties by comprehending elements of economy, politics and sociology. Based on the example of La Crau, a steppe area in South-eastern France, this review aims at (i) illustrating that this guideline can facilitate creating and managing protected areas and (ii) proposing improvement to the guideline while keeping it simple. In La Crau, the need for conservation was first acknowledged in 1975. Between 1983, when a request for a protection decree was made and 1990, when the first concrete protection measure was taken, 20% of the steppe disappeared. It took another 8 years to reach a concerted management plan in 1998. The review shows how using ecological guidelines would have helped better and faster protection of the steppe. Improvements to the guideline drawn from the La Crau experience include: the protection of some traditional practices; the protection of some degraded habitats that can substitute for habitats that no longer exist; the restoration of degraded habitats that do not qualify for protection; and the flexibility of the management plan. 相似文献
7.
Brownfields that are reused for the generation of renewable energy are called brightfields. This rapidly emerging idea advocates the combination sustainable site reuse and the generation of electricity from renewables. While programmes like the RE-Powering America's Land Initiative herald its benefits, academia knows but little of its barriers and challenges. This study aims to examine the technical/environmental, regulator/financial/institutional, and social barriers to this type of development, along with measures that may address them. The barriers and measures were predominately identified via a qualitative and quantitative survey sent to brightfield developers (experts), along with a review of case studies and the emerging brightfield literature. The study found that environmental/technical barriers only differ from ‘conventional’ renewable energy projects (e.g. on Greenfields) in the event of site contamination. The latter then is what makes brightfields unique and is the source for a myriad of challenges concerning risk and liability, which cause financial problems and investment hesitance. However, evidence conjectures that there is less contextual public opposition to brightfields compared to conventional renewable energy. 相似文献
8.
Benjamin Erable Isabelle GoubetAmira Seltana Thierry Maugard 《Journal of environmental management》2009
Traditional biological removal processes are limited by the low solubility of halogenated compounds in aqueous media. A new technology appears very suitable for the remediation of these volatile organic compounds (VOCs). Solid/gas bio-catalysis applied in VOC remediation can transform halogenated compounds directly in the gas phase using dehydrated cells as a bio-catalyst. 相似文献
9.
Relationships between street characteristics and perceived attractiveness for walking reported by elderly people 总被引:2,自引:0,他引:2
Hieronymus C. Borst Henk M.E. Miedema Sanne I. de Vries Jamie M.A. Graham Jef E.F. van Dongen 《Journal of environmental psychology》2008,28(4):353-361
Walking is important for the health of elderly people. Previous studies have found a relationship between neighbourhood characteristics, physical activity and related health aspects. The multivariate linear regression model presented here describes the relationships between the perceived attractiveness of streets for walking along and (physical) street characteristics. Two hundred and eighty-eight independently living elderly people (between 55 and 80 years old) participated in the study. Street characteristics were assessed along homogeneous street subsections defined as ‘links’. Positively related to perceived attractiveness of links were the following street characteristics: slopes and/or stairs, zebra crossings, trees along the route, front gardens, bus and tram stops, shops, business buildings, catering establishments, passing through parks or the city centre, and traffic volume. Litter on the street, high-rise buildings, and neighbourhood density of dwellings were negatively related to perceived link attractiveness. Overall, the results suggest that three main aspects affect perceived attractiveness of streets for walking, namely tidiness of the street, its scenic value and the presence of activity or other people along the street. The results are discussed within the context of these three aspects. 相似文献
10.
Assia Kaci Fabienne Petit Patrick Lesueur Dominique Boust Anne Vrel Thierry Berthe 《Environmental science and pollution research international》2014,21(18):10787-10802
In estuarine ecosystems, trace metals are mainly associated with fine grain sediments which settle on mudflats. Over time, the layers of sediments accumulate and are then transformed by diagenetic processes, recording the history of the estuary’s chemical contamination. In such a specific environment, we investigated to what extent a chronic exposure to contaminants could affect metal-resistant sedimentary bacteria in subsurface sediments. The occurrence and diversity of cadmium resistance genes (cadA, czcA) was investigated in 5- and 33-year-old sediments from a highly contaminated estuary (Seine France). Primers were designed to detect a 252-bp fragment of the czcA gene, specifically targeting a transmembrane helice domain (TMH IV) involved in the proton substrate antiport of this efflux pump. Although the cadA gene was not detected, the highest diversity of the sequence of the czcA gene was observed in the 5-year-old sediment. According to the percentage of identity at the amino acid level, the closest CzcA relatives were identified among Proteobacteria (α, β, γ, and δ), Verrucomicrobia, Nitrospirae, and Bacteroidetes. The most abundant sequences were affiliated with Stenotrophomonas. In contrast, in the 33-year-old sediment, CzcA sequences were mainly related to Rhodanobacter thiooxydans and Stenotrophomonas, suggesting a shaping of the metal-resistant microbial communities over time by both diagenetic processes and trace metal contamination. 相似文献