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Pfeiffer syndrome is an autosomal dominant disorder characterized by coronal craniosynostosis, midface hypoplasia, broad thumbs and great toes. On the basis of clinical findings, three subtypes have been delineated. The clinical variability of Pfeiffer syndrome as well as other causes of craniosynostosis can make a prenatal diagnosis based on sonography alone difficult. We describe a fetus in whom sonographic findings (including 3D ultrasound) suggested a Pfeiffer syndrome type II and in which subsequent molecular analysis verified the diagnosis by identifying a de novo mutation in the FGFR2 gene. To the best of our knowledge, this is the first report of a prenatal molecular diagnosis of Pfeiffer syndrome in a patient without family history. Copyright © 2004 John Wiley & Sons, Ltd.  相似文献   
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Livestock production today faces thedifficult task of effectively meeting emergingconsumer concerns while remaining competitive on majortarget markets. Meeting consumer concerns aboutproduct safety and animal welfare are identified askey attention points for future livestock production.The relevance of these issues pertains to productionefficiency and economic benefits and tore-establishing meat sector image and consumer trust.The current paper analyses consumer concerns about theethical issues of meat safety and animal welfare fromcurrent livestock production. The research methodologyis based on literature review, secondary data sources,and primary research through focus group discussionsand a survey of 320 meat consumers in Belgium.Objectives were to assess importance attached byconsumers to product safety and animal welfare asethical issues in commercial livestock production, andto evaluate consumer perception of these issues forbeef, pork, and poultry. Significant differences inissue importance and perception are identified amongconsumer groups based on socio-demographic andbehavioral characteristics. From the analysis, meatsafety emerges as an absolute but minimum requirementfor future success of livestock and meat production.Additionally, animal welfare can be expected to becomea critical theme especially for pork and poultryacceptance.  相似文献   
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Specifying information needs: improving the working methodology   总被引:1,自引:1,他引:0  
Specification of information needs is an important step in the design of monitoring networks. Within the framework of Monitoring Strategy 2000+, a programme of the Dutch Directorate General of Public Works and Water Management (Rijkswaterstaat) which seeks to provide innovation in the monitoring sector, a method has been developed to link water management policy to monitoring through specification of information needs. Over the past 2 years, this method has been applied in several projects within the Rijkswaterstaat organisation. Use of this method has led to improvements in the monitoring practice and was judged very positively by the people involved. Nevertheless, the main obstacle to employing the method was the actual translation of policy matters into information products. A special study is carried out, focusing on this aspect within the method, to provide the participants of a project with a mental framework that enables them to clearly specify their information needs. The major requirements for this mental framework are that all participants have a clear view of the process (system), and that they are able to relate their contribution to this process and to the contributions of others (transparency). The first step of the process is to focus the attention of stakeholders on the information they will actually need for decision-making. The next step is to confront the different expressed opinions through the method of the 'devil's advocate', which implies criticising the results of the first step by finding as many as possible arguments against these opinions. This should force the stakeholders to better specify their arguments. The method, its improvements and the results of one pilot project will be discussed in this paper. Electronic Publication  相似文献   
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Lindane (??-hexachlorocyclohexane, ??-HCH) is a recalcitrant and toxic organochlorine insecticide. Due to its non-selective production process and widespread use, HCH isomers and their degradation products have been detected frequently in soils and groundwater. An innovative technology using microbial produced Pd(0) nanoparticles, i.e. bio-Pd, was developed to treat groundwater containing a mixture of HCHs and chlorobenzenes. In a first step, the groundwater was de-ironized and most of the chlorobenzenes were removed in a biological trickling filter. The ??g?L?1 levels of HCHs and chlorobenzenes were removed in a second step by the bio-Pd-based technology. Therefore, a 200-L pilot scale reactor was developed with 100?mg?L?1 bio-Pd encapsulated in alginate beads. Hydrogen gas was bubbled at the bottom of the reactor and served to charge the bio-Pd catalyst. The reactor influent contained 5.2???g?L?1 HCHs and 51.1???g?L?1 chlorobenzenes. During a test period of 10?days, 29% of the HCH isomers and 63% of the chlorobenzenes were removed applying a nominal hydraulic residence time of 4?h. These removal percentages could be increased to 75 and 68% by doubling the nominal hydraulic residence time to 8?h. This study demonstrated that biologically produced nanoparticles of Pd can be applied for the large-scale remediation of groundwater contaminated with HCHs.  相似文献   
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The awareness of the problem of the scarcity of water of high quality has strongly changed the approach of wastewater treatment. Currently, there is an increasing need for the beneficial reuse of treated wastewater and to recover valuable products and energy from the wastewater. Because microbiological treatment methods are, only to a limited part, able to satisfy these needs, the role and significance of physical/chemical processes in wastewater treatment are gaining more and more interest. The specific future role and aim of the various physical/chemical treatment processes can be categorized in five groups: improvement of the performance of microbiological treatment processes, achievement of the high quality required for reuse of the effluent, recovery of valuable components and energy from the wastewater for beneficial reuse, desalination of brackish water and seawater, and treatment of concentrated liquid or solid waste residues produced in a wastewater treatment process. Development of more environmentally sustainable wastewater treatment chains in which physical/chemical processes play a crucial role, also requires application of process control and modeling strategies. This is briefly introduced by the elaboration of treatment scenarios for three specific wastewaters.  相似文献   
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Rotary kiln incinerators and cement kilns are two energy intensive processes, requiring high temperatures that can be obtained by the combustion of fossil fuel. In both processes, fossil fuel is often substituted by high or medium calorific waste to avoid resource depletion and to save costs. Two types of industrial calorific waste streams are considered: automotive shredder residue (ASR) and meat and bone meal (MBM). These waste streams are of current high interest: ASR must be diverted from landfill, while MBM can no longer be used for cattle feeding. The environmental impact of the incineration of these waste streams is assessed and compared for both a rotary kiln and a cement kiln. For this purpose, data from an extensive emission inventory is applied for assessing the environmental impact using two different modeling approaches: one focusing on the impact of the relevant flows to and from the process and its subsystems, the other describing the change of environmental impact in response to these physical flows. Both ways of assessing emphasize different aspects of the considered processes. Attention is paid to assumptions in the methodology that can influence the outcome and conclusions of the assessment. It is concluded that for the incineration of calorific wastes, rotary kilns are generally preferred. Nevertheless, cement kilns show opportunities in improving their environmental impact when substituting their currently used fuels by more clean calorific waste streams, if this improvement is not at the expense of the actual environmental impact.  相似文献   
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