Prenatal diagnosis of the Hunter syndrome and the introduction of a new fluorimetric enzyme assay

Prenatal diagnosis of the Hunter syndrome (mucopolysaccharidosis type II; MPS II) is preferably achieved by the assay of iduronate-2-sulphate sulphatase (IDS) in uncultured chorionic villi (CV) as this allows early (12th week), rapid (2–3 days) and reliable results. We summarize the results of 174 prenatal analyses in the past 30 years, using various methods such as radiolabelled sulphate incorporation in amniotic fluid (AF) cells, glycosaminoglycan (GAG)-electrophoresis in AF and IDS assay in CV, CV-cells, AF and AF-cells. Twenty-seven fetuses with MPS II were diagnosed after finding clearly abnormal results in pregnancies with a male fetus; very low IDS activity has also been measured in some pregnancies with a (heterozygous) female fetus, emphasizing the need to combine enzyme assay with fetal sex determination. IDS activity has until recently been assessed by a cumbersome radioactive enzyme assay. Here we describe the use of a novel fluorigenic 4-methylumbelliferyl substrate, which allows a sensitive, rapid and convenient assay of IDS activity and reliable early prenatal diagnosis. This novel IDS assay was validated in retrospective analyses of 14 CV, CV-cell, AF and AF-cell samples from affected pregnancies in addition to prospective prenatal diagnosis in eight pregnancies at risk with one MPS II-affected fetus. Copyright © 2002 John Wiley & Sons, Ltd.     

NitroGenius:氮决策支持系统--荷兰氮污染问题最佳解决方案的一个游戏模拟实验

利用计算机模拟的方法,我们为第二届国际氮会议研制开发了一个游戏形式的氮决策支持系统.研制这一系统的目的是:①使科学工作者和决策者们都能认识到荷兰氮污染问题的复杂性,因为荷兰是一个农业、工业和运输业活动密集的区域;②探索以最低的社会经济代价解决氮污染的最佳方案.NitroGenius由有关时空范围内的氮流动模型组成,其中包括氨和氮氧化合物的释放及其对地表水和地下水的污染.NitroGenius中还包括了一个经济模型,它描述了各个重要经济部门之间的关系,以及不同氮排放控制措施对国民生产总值(GDP)、失业率、能源消耗和环境的影响.在第二次国际氮会议期间,大约有50个研究组对NitroGenius进行了测试,结果表明,如果认真进行计划并选择适当的治理措施,就能够以适当的代价解决荷兰的氮污染问题.     

Institutional dynamics and climate change adaptation in South Africa

Climate change is a multi-dimensional issue and in terms of adaptation numerous state and non-state actors are involved from global to national and local scales. The aim of this paper is first to analyse specific institutional networks involved in climate change predominantly at the national level in South Africa and second to determine how different stakeholders perceive their role vis-a-vis climate change adaptation. Within the South African context there is a gap in understanding and evaluating how institutional networks operate and thus the findings of this work may help inform and strengthen such relationships in the future. Results showed that few institutions fully understand the implications of adaptation and their roles and responsibilities have not yet been properly defined. Constraints relating to capacity, lack of awareness and poor information flow need to be addressed. Climate change is perceived as an important issue although problems such as poverty reduction and job creation remain national priorities. Most importantly this research has demonstrated how adaptation challenges the hierarchical manner in which government works and a more collaborative approach to climate change adaptation is needed. Adaptation needs to be mainstreamed and institutional networks need to be strengthened in order for adaptation mechanisms to be effectively implemented.

地下水铀污染的原位微生物还原与固定:在美国能源部田纳西橡树岭放射物污染现场的试验

总结了美国斯坦福大学和橡树岭国家实验室等在美国能源部田纳西州橡树岭综合试验基地进行的铀污染原位微生物修复阶段性试验结果.本试验利用微生物以乙醇为电子供体还原地下水和沉积物中的六价铀为不溶解的四价铀,使之原位固定化.随后通过加入溶解氧和硝酸盐来试验微生物还原后的地下水层中还原固定态铀的稳定性.通过预处理和长期间隔注入乙醇...