Ecological knowledge and North Sea environmental policies

In an analysis of North Sea eutrophication science and policies, focusing on the period 1980–2005, it was investigated how scientific information was used in policy-making. The analysis focused on the central assumptions of the rational policy-making model, i.e. that scientific information can be used to formulate decisions, based upon objective scientific information (rational decision-making), and secondly, can support implementing these decisions (rational management). In general terms, the following was concluded:

• •More knowledge has increased rather than reduced uncertainty;
• •In order to handle the problem of dealing with complexity and uncertainty at the political level, a simplification of facts has occurred, in this case focusing on nutrients as the main cause of the problem, at the same time excluding other possible causes;
• •Both the limited scientific view (i.e. the nutrient view) and the exaggeration of the seriousness of the problem (impacts, scope) have been used as an authoritative basis for the justification of political decisions. Both were not supported by the majority of the scientific community;
• •New scientific knowledge, not in support of existing policies, has been excluded from the policy process;
• •The science–policy interface, mainly consisting of “civil-servant scientists”, that emerged and increased its influence over the period of investigation, has been the central element in the simplification and exclusion process.

The main lesson learned is that work at the interface of science and policy must be subject to democratic principles, i.e. be transparent and involving all parties with a stake in the issue under consideration.     

Germline mosaicism complicates molecular diagnosis of Lesch–Nyhan syndrome

A healthy female with a brother suffering from Lesch–Nyhan syndrome was assigned a carrier status on the basis of haplotype analysis employing flanking and intragenic polymorphic markers of the HPRT gene. Her mother has been confirmed as a definite carrier by cell growth selection studies in cultured fibroblasts. In our proposita's first pregnancy, a male fetus was identified carrying the risk allele. Afterwards, the underlying novel mutation A161E (GCA→GAA at position c482) could be identified in the affected brother and in the heterozygous mother but not in the DNA of the pregnant sister and fetus. The fetus was also confirmed to be normal by uptake of 14C-hypoxanthine in cultured amniotic cells. To test the discrepancy, the investigation was extended by recruiting additional family members. The data obtained showed that the mother had passed her risk haplotype to the affected son as well as to her mutation-carrying and non–mutation-carrying daughters. This provides the first evidence of concomitant somatic and germline mosaicism in Lesch–Nyhan syndrome. The study has a bearing on genetic counselling and cautions against the reliability of only using indirect genetic diagnosis even with intragenic markers. Copyright © 2004 John Wiley & Sons, Ltd.     

新常态下如何实现环境保护的纳什均衡

从自然资源和生态环境的公共性出发,认为以经济建设为中心的政策选择,促成了以淡视生态环境为前提的均衡策略,让经济发展与环境保护之间形成"坏"的纳什均衡,而新常态能够为经济发展和环境保护之间达成好的纳什均衡提供了改革保障、法制保障和经济保障,因此,新常态下的生态文明建设能够解套环境保护的"囚徒困境",最重要的任务是借助政府这一外力制定新的"游戏规则",以打破原有的"纳什均衡",通过建立健全环境成本内部化的政策措施和生态补偿机制、改革政绩考核机制实现对绿色发展的激励等措施,推进经济发展与环境保护之间达成理想的"纳什均衡"。     

Prenatal diagnosis of Schwartz–Jampel syndrome with early manifestation

A mother who had given birth to a child with Schwartz–Jampel syndrome (SJS) with neonatal manifestations (myotonia, congenital contractures, bowing of femora and tibiae) underwent ultrasonic fetal examination during the 17th and 19th week of her second pregnancy. Moderately decreased fetal motor activity and constant flexion of the fingers were observed at both examinations. In addition, there was mild bowing and shortening of the femora. At birth, the child presented with the characteristic pattern of SJS similar to her older brother. Prenatal ultrasonic diagnosis of Schwartz–Jampel syndrome is possible, at least for the form with neonatal onset of myotonia and contractures.     

Meckel–Gruber syndrome: Ultrasonographic diagnosis at 13 weeks' gestational age in an at-risk case

A case of early diagnosis at 13 weeks' gestational age of Meckel–Gruber syndrome by ultrasound is reported in a patient with a 25 percent recurrence risk. The usefulness of genetic counselling and aimed echographic examination is discussed.     

A Bayesian network highlighting the linkages between landscape structure and the local economy: the case of agritourism in lowland areas of Northern Italy

Linking landscapes to socio-economic benefits necessarily requires considering the usability of landscape structure. To do so, however, depends on the interaction between users and producers of landscape-related services. We illustrate this interaction with a Bayesian Belief Network (BBN) in a case study analysing the connection between residents' perceptions of landscape structure and agritourism restaurants in the eastern lowlands of Ferrara (Italy). We use estimates of prior and conditional probabilities from a mix of different data: land use, survey data, regional statistics, and expert judgements to show the likely effects of the landscape structure on the local economy by using intermediate forms of services (i.e. second-order services). The second-order service is highly influenced by the agritourism density and by the frequency with which customers dine at agritourism restaurants and less by landscape attractiveness, confirming the importance of the supply and demand of second-order services in the provision of landscape-related services.     

Reuse of nickel recovered from spent Ni–Cd batteries for the preparation of C/Ni and C/Ni/Pd layered electrodes for energy sources

Nickel recovered in the recycling process of Ni–Cd batteries was used as a main component of bath for electrodeposition of nickel onto carbon fabric in order to obtain C/Ni composite. A part of the obtained composite was additionally coated with a thin layer of palladium (C/Ni/Pd composite). All the materials were investigated to assess the possibility of their use in the processes of electrochemical storage (sorption/desorption) of hydrogen and electrochemical oxidation of methanol. The obtained results showed the composites to be active electrode materials in these processes. The electrodes exhibited high activity and cyclability, especially in the process of methanol oxidation due to electrocatalytic activity of both nickel deposited onto carbon fabric and the outer layer of porous palladium coating nickel substrate.     

Prenatal diagnosis of a new syndrome: Holoprosencephaly with hypokinesia

Markedly decreased fetal activity (akinesia/hypokinesia) is usually readily apparent to experienced mothers, and frequently this concern leads to attempts at prenatal diagnosis. We report prenatal diagnosis of two fetuses with congenital contractures, markedly decreased fetal movement, and microcephaly due to severe holoprosencephaly. Such familial recurrence to phenotypically normal parents suggests a newly recognized autosomal recessive or X-linked syndrome that is readily detectable by prenatal ultrasonography.