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.     

Prenatal diagnosis of genetic microcephaly

True microcephaly can be diagnosed at an early stage of gestation by serial measurements of fetal head growth as demonstrated by this case report in which the diagnosis of genetic microcephaly was made but termination refused. True microcephaly was evident at birth.     

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.     

Design of policy mechanism to promote cleaner production in China

ＩｎｔｒｏｄｕｃｔｉｏｎＣｌｅａｎｅｒｐｒｏｄｕｃｔｉｏｎ (ＣＰ)ｈｏｌｄｓｇｒｅａｔｐｒｏｍｉｓｅａｓａｎｅｆｆｅｃｔｉｖｅｓｔｒａｔｅｇｙｉｎｔｈｅｆａｃｅｏｆｄｏｕｂｌｅｃｈａｌｌｅｎｇｅｓｏｆｅｎｖｉｒｏｎｍｅｎｔａｌｐｒｏｔｅｃｔｉｏｎａｎｄｅｃｏｎｏｍｉｃｄｅｖｅｌｏｐｍｅｎｔ.Ｉｎ 1993,ａｄｅｍｏｎｓｔｒａｔｉｏｎｐｒｏｊｅｃｔ (Ｂ 4ｐｒｏｊｅｃｔ) ,ｄｅｓｉｇｎｅｄａｎｄｅｘｅｃｕｔｅｄｂｙｔｈｅＳｔａｔｅＥｎｖｉｒｏｎｍｅｎｔａｌＰｒｏｔｅｃｔｉｏｎＡｄｍｉｎｉｓｔｒａｔｉｏｎ (…