Prenatal diagnosis of the hurler syndrome: Report on 40 pregnancies at risk

In 40 pregnancies at risk for the Hurler syndrome 13 affected fetuses were detected by the demonstration of an α-L -iduronidase deficiency and an increased level of ³⁵S-sulphate incorporation. The diagnoses were confirmed by the analysis of fetal tissues and/or cultured fetal skin fibroblasts. Microassays for α-L -iduronidase, using phenyl α-L -iduronide and more recently 4–methyl-umbelliferyl α-L -iduronide, enabled a reliable diagnosis to be made within 15 to 18 days after amniocentesis. ³⁵S-sulphate incorporation has been a valuable adjunct in cases with a low (heterozygote) enzyme activity.     

Application of the analytic hierarchy process to select characterization and risk-based decision-making and management methods for hazardous waste sites

Environmental managers at U.S. Department of Defense (DoD) installations overseas are faced with the challenge of managing contaminated sites at these installations with little information on the extent of contamination or the risk posed by the site. In this regard, DoD managers overseas encounter a situation quite similar to the situation faced by decision makers in the U.S. who are managing brownfields. Innovative site characterization and risk-based decision-making methods, which are currently being developed for expeditious application at brownfield sites in the U.S., may also be appropriate for application at overseas DoD sites. In this paper, the analytic hierarchy process (AHP) is used by DoD decision makers to evaluate and rank innovative site characterization technologies and risk-based decision-making and management methods, for use at installations in Korea. Results indicate that for sites with high potential risk the decision makers preferred site characterization technologies that produce data of high quality and a method that can be used to establish credible risk-based remediation goals. This study provides a framework for applying characterization technologies and risk management to poorly characterized contaminated sites in developing countries, where resources for remedial actions may be limited.     

北方高纬度地区生态系统动态、生物多样性管理及社会政策

由于受到众多因素大规模作用的影响,高纬度地区生态系统的变化异常活跃.这些地区的生态系统非常脆弱,各种全球变化过程都在影响着可更新林木资源持续生产和当地社会赖以生存的动植物资源丰度.本文对于北半球高纬度地区生态系统的一些新认识及其(所包含的)意义进行了讨论,并对提高生态系统的恢复力所采取的必要管理措施进行了探讨.认为在生态系统面临各种变化和干扰时系统管理的焦点应该从系统的恢复转移到系统功能的维系上来.生物多样性的作用是确保生态系统在经历干扰和重组时能够进行重组和发展,这一作用应该在系统管理与相关的方针政策中得到重视.强调应重新考虑现在的生态保护区的概念以发展一些动态的管理方法,使生态系统在面临环境变化时能够进行可持续的管理.而高纬度地区一些土著人习惯性的生态保护区的特点与那些对保护区进行动态保护的观点往往是一致的.针对高纬度地区动态景观中的生物多样性管理我们提出了新的发展方向,并从非传统的观点方面为其提供了经验例证.这些非传统的观点与看法可能会有助于提高生物多样性可持续管理和生态系统功能发挥的潜力.