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We report the prenatal diagnosis at 16 weeks' gestation of bilateral split-hand/split-foot malformation (SHSFM) with severe lobster claw deformity of hands and feet in a male fetus without associated malformations. A minor manifestation of SHSFM was present in the father with only mild bilateral foot involvement (syndactyly I–II; cleft II–III; left cutaneous syndactyly III–IV). Mutation analysis of the p63 gene on chromosome 3q27 showed a missense mutation 577A→G (predicting amino acid substitution K193E) in the father. This mutation has not been reported so far in SHSFM but resembles the previously reported 580A→G (predicting amino acid substitution K194E) in a family with SHSFM. Copyright © 2001 John Wiley & Sons, Ltd.  相似文献   
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The very significant impact of European legislation (Directive 91/414/EEC) on the authorization of plant protection products is reviewed herein, which has resulted in withdrawal of 704 active substances (AS) out of 889 assessed so far. The list of currently approved 276 AS includes 194 AS “existing” in the market before 1993 and 82 “new” AS introduced during the last 15 years. Results of toxicity characterization of the approved AS are also summarized, utilizing several well-known databases. Although significant data gaps exist for a rather large part of the approved AS, it is found that 84 AS are positive for at least one health effect (after chronic and/or acute exposure) including carcinogenicity, reproductive and neuro-developmental disorders, as well as endocrine disruption. The toxicity characterization results of this study are compared to those of recent assessments by other organizations (KemI, the Swedish Chemicals Agency, and the Pesticide Safety Directorate of the UK), where interpretation and use is made of AS “cut-off” criteria foreseen in new EU legislation. These studies report a comparatively smaller AS number with positive toxicity characterization. The possibility of some additional AS withdrawal in the near future, combined with the rather small rate of new AS introduction (approx. 5 per year) suggest that the list of approved AS over the next 10–15 years may not change very drastically. Consideration of the above trends is necessary and instructive in evaluating results of existing health impact assessment (HIA) studies, as well as in planning new ones. Due to the very drastic change in the number and type of marketed AS, that took place within the past 8–9 years, it is suggested that new HIA studies (based on epidemiological data after year 2000) should focus on a rather short time frame and, therefore, on appropriate cohort groups, e.g. young children. For the same reason, results of epidemiological studies of the past (involving banned AS) should be carefully interpreted and used with caution.  相似文献   
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The EEC Shellfish Directive is a policy designed to protect and, where necessary, improve the quality of designated shellfish waters. Its implementation within the UK, however, has had no effect upon water quality for two reasons. First, the policy has important defects, having ambiguities concerning public health provisions and lacking designation criteria. Second, UK government has sought to achieve formal compliance, while at the same time ensuring that its full financial impact on public expenditure has been contained. Consequently, only those fisheries which already comply with water quality standards have been designated. Within Wales, one fishery has been designated, while other, commercially more important, but grossly contaminated shellfisheries have not.  相似文献   
4.
Material recovery processes are presented as the optimum option for recycling plastic wastes as a means of recovering hydrocarbon resources. There exist a large variety of automated material recovery processes for recycling of such wastes but each with significant limitations. Of these, the separation based on differences in densities is advocated as the optimum process either for producing recycled products or preparing wastes for subsequent recovery processing.Density separation processes based on cyclone type density media separation (DMS) is presented as an important, potential method for increasing plastics recycling process capacities. It is demonstrated to have the capacity to separate a significantly larger range of particle sizes than those presently processed industrially. The mathematical relationship for the prediction of quality of typical LARCODEMS type density media separations by particle size and density as expressed by the Ecart Probable is presented.A proposed device configuration is presented for density media separation to optimize the recovery and purity of both density fractions produced. It is also suggested that to be economically viable, a large scale of operation is required for industrial plastics recycling operations recovering and producing a number of different plastics with a purity to be used as a substitute for virgin material.  相似文献   
5.
Environmental problems are a function of the process of economic development and the ability to mitigate the problems through regulation. An historical analysis helps to show how the problems have been solved thus far. The objective of this investigation is to present an overview of environmental problems and the corresponding regulations in Western Europe during the 1980s. Environmental problems for 18 Western European countries were identified from abstracts from a data base on environmental literature. The 1977 findings were systematized on the basis of 20 key words according to source and impact, and a new data base was established. The data showed that the main environmental problems in Western Europe were: energy production/acid rain, nuclear power/waste, air pollution/climate, heavy metals, pesticides, water pollution, solid waste, and unforeseen accidents. Many environmental problems are common to most European countries. Regulatory measures differ significantly. In some countries environmental regulations are largely limited to the implementation of European Economic Community (EEC) directives. We have supplemented the national analyses with an examination of existing and proposed EEC regulations along with interviews with civil servants in the EEC Commission. In the 1980s, EEC environmental regulations were similar to environmental regulations in some member states. It is expected that future EEC regulations will be less reflective of those of individual member states.  相似文献   
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Background, aim, and scope  Dicofol is widely used as a pesticide in agriculture applications. Since dicofol is mainly synthesized from dichlorodiphenyltrichlorethane (DDT), it contains DDT as an impurity. The European Community has forced Prohibition Directive 79/117/EEC to reduce DDT in dicofol formulations. Specifically, DDT content in a dicofol formulation cannot exceed 0.1%. The goal of this project was to determine the DDT content in dicofol formulations used in Turkey. Materials and methods  Samples of all the dicofol formulations in Turkey were collected to quantify DDT and DDT-related compounds. Four replicates were used for each sample. GC/MS/MS was used to analyze p,p′ and o,p′ isomers of DDT, DDD, and DDE. A HPLC was used to determine p,p′-Cl-DDT concentrations. Results  The total DDT content of the formulated dicofol was found between 0.3% and 14.3%. The concentration of p,p′-DDE ranged from 167 to 1,042 mg kg−1 in dicofol samples. p,p′-DDT concentrations were found to be 32 to 183 mg kg−1. The o,p’-DDT level ranged from 2 to 34 mg kg−1 in the dicofol formulations analyzed. Discussion  It was estimated that 617.8 kg of DDT was released from dicofol. The main impurity was identified as p,p-Cl-DDT. Based on these results, dicofol serves as a continuing source of DDT contamination. Conclusions  All DDT concentrations in dicofol samples analyzed were higher than the permitted 0.1% level of Prohibition Directive 79/117/EEC. The reduction of dicofol is critical since it serves as a continual source of DDT contamination. Recommendations and perspectives  DDT has been found in soil, water, and air samples. Dicofol has been identified as a contributor to continued DDT contamination in soil and water. More studies are needed to ascertain the source of DDT in the air.  相似文献   
8.
Transvaginal ultrasonography performed at 14 weeks' gestation demonstrated a bilateral cleft lip and lobster-claw deformities of the hands and feet, in keeping with the diagnosis of EEC syndrome (ectrodactyly or lobster-claw deformity, ectodermal dysplasia, and cleft lip and palate). The fetus was aborted and the diagnosis of EEC syndrome was confirmed.  相似文献   
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