Prenatal diagnosis of NADH:ubiquinone oxidoreductase deficiency

NADH:ubiquinone oxidoreductase (complex I of the mitochondrial respiratory chain) deficiency is a severe disorder with an often early fatal outcome. Prenatal diagnosis for complex I defects currently relies mainly on biochemical assays of complex I in fetal tissues such as chorionic villi (CV), and is only in a minority of cases possible by means of mutational analysis of nuclear-encoded genes of complex I. We report on our experience to date with prenatal diagnosis in pregnancies at risk for complex I deficiency. We measured complex I activity in native CV and/or cultured CV in 23 pregnancies in 15 families. In accordance with the results of the investigations in CV, 15 children were born clinically unaffected. Two prenatally diagnosed unaffected fetuses and two prenatally diagnosed affected fetuses were lost prematurely with spontaneous or provoked abortions, respectively. Two affected children were born (prenatally found to be affected). In two pregnancies a discrepancy between native and cultured cells was found. We conclude that prenatal diagnosis for complex I deficiency can be reliably performed. Pitfalls were encountered in using cultured CV as a result of maternal cell contamination (MCC). Future research on pathogenic nuclear mutations underlying complex I deficiency will extend the possibilities for prenatal diagnosis at the molecular level. Copyright © 2001 John Wiley & Sons, Ltd.     

Split-hand/split-foot malformation with paternal mutation in the p63 gene

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.     

职业安全健康管理体系在冶金建筑施工企业中的应用

通过对冶金建筑施工企业伤亡事故现状的分析，提出建立职业安全健康管理体系的意义和重要性，最后指出冶金建筑施工企业在建立职业安全健康管理体系时存在的问题及建议。     

浅谈海上油井安全阀及其控制系统技术的应用

针对实际生产条件，为全面推行海上安全环保、节能与清洁生产，在油井上安装了太阳能安全阀控制系统，对油井生产与安全管理起着重要的作用。     

循环式活性污泥法中厌氧生物选择区的运行条件研究

研究了循环式活性污泥法中厌氧生物选择区的停留时间、进水有机物浓度和系统中泥龄、曝气时间等运行参数对选择区内厌氧释磷以及有机物的吸收贮存的影响。结果表明，选择区停留时间lh，系统泥龄5d，曝气2h，进水有机物浓度COD／TP=60--70，选择器中释磷效果及微生物的吸附性能最优，可去除进水中78％的COD。而且污泥沉降性能良好，试验运行过程中，SVI一直保持在30--40ml／g左右。     

植物根系对镉毒害的防御及其代谢

本文采用植物根系生物量、根系内活性氧清除酶活性变化和植物表观中毒症状作指标 ,研究了 Cd毒害对草坪植物的毒性作用、根系应激反应。结果表明 ,当土壤溶液中 Cd浓度达 1.5× 10 - 3mol· dm- 3时 ,植物体内活性氧代谢、生长状况将出现异常 ,甚至出现明显的表观中毒症状。 Cd对植物体内清除酶活性的毒性强度为 CAT>SOD>POD。匍匐翦股颖 (Agrostis stolonifera)根系对 Cd毒害的抵抗作用强于多年生黑麦草 (L olium perernne)。     

Brazil's Electric Power Choices and Their Corresponding Carbon Emissions Implications

This study analyzes the options for meeting power demand in the Brazilianpower sector through the year 2015. Three policy cases are constructedto test economic and environmental policy measures against a baseline:advanced technologies scenario, environmental control scenario and carbon(C) elimination scenario. Least-cost modeling simulated these scenarios throughchanges in emissions fees and caps, costs for advanced technologies,demand side efficiency, and clean energy supplies. Results show that, in theabsence of alternative policies, new additions to Brazil's electric powersector will shift rapidly from hydroelectricity to combined-cycle natural gasplants. When the cost of environmental impacts are incorporated in theprice of power, the least-cost mix of electric power generation technologycould change in other ways. In all scenarios, energy efficiency andcogeneration play an important role in the least-cost power solution. Savingelectricity through increased efficiency offsets the needs for new supply andhas enormous potential in Brazil's industrial sector. Efficiency also reducesthe environmental burden associated with electricity production andtransmission, without compromising the quality of the services demandedby end users. Interesting enough, carbon dioxide (CO₂) emissions will remainrelatively low under almost every conceivable scenario.     

Multimedia model of atrazine in plant-soil-groundwater system with a fugacity approach

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焦作市城市社会生态环境评价

在调查研究的基础上，针对焦作市2000年的城市生态环境现状，构建了城市社会生态环境质量评价体系，由3个层次24个指标组成。对焦作市城市社会生态环境进行单因素和综合质量评价。评价结果表明，该市的城市社会生态环境质量比较差，并存在一些主要社会生态环境问题。最后，针对性地提出了一些改善治理的对策。     

人工神经元网络对水处理系统建模适应性的研究

针对水处理系统的特点和研究中的难点，引入人工神经元网络的理论和思想，提出了水处理系统的神经网络分析方法．通过建立基于ＢＰ人工神经元网络的臭氧生物活性炭系统的模型，具体地考察了人工神经元网络对水处理系统建模的适应性，使水处理系统的研究迈向智能化和控制化．