The cerebro-hepato-renal (Zellweger) syndrome: Prenatal detection based on impaired biosynthesis of plasmalogens

Prenatal diagnosis of the cerebro-hepato-renal (Zellweger) syndrome has been performed in 10 pregnancies at risk by measuring both the activity of acyl CoA: dihydroxyacetonephosphate acyltransferase (DHAP-AT) and the de novo plasmalogen biosynthesis, either in cultured amniotic fluid cells or in fibroblasts cultured from a chorionic villus biopsy. In 7 of the pregnancies both tests indicated no abnormality. All 7 continued to term and normal infants were delivered. However, in amniotic fluid cells from 2 fetuses affected by Zellweger syndrome unequivocal differences from control values were found. The activity of DHAP-AT was clearly deficient and the de novo plasmalogen biosynthesis was impaired. In one pregnancy at risk prenatal diagnosis was performed during the first trimester by measuring both the DHAP-AT activity and the de novo plasmalogen biosynthesis in fibroblasts cultured from a chorionic villi biopsy. From the deficient DHAP-AT activity and the impaired de novo plasmalogen biosynthesis it was concluded that the fetus was affected. This was confirmed biochemically after induced abortion. It can be concluded that measurement of the DHAP-AT activity and the de novo plasmalogen biosynthesis provides convenient methods for the early prenatal detection of Zellweger syndrome.     

Chemical Leasing business models and corporate social responsibility

Chemical Leasing is a service-oriented business model that shifts the focus from increasing sales volume of chemicals towards a value-added approach. Recent pilot projects have shown the economic benefits of introducing Chemical Leasing business models in a broad range of sectors. A decade after its introduction, the promotion of Chemical Leasing is still predominantly done by the public sector and international organizations. We show in this paper that awareness-raising activities to disseminate information on this innovative business model mainly focus on the economic benefits. We argue that selling Chemical Leasing business models solely on the grounds of economic and ecological considerations falls short of branding it as a corporate social responsibility initiative, which, for this paper, is defined as a stakeholder-oriented concept that extends beyond the organization’s boundaries and is driven by an ethical understanding of the organization’s responsibility for the impact of its business activities. For the analysis of Chemical Leasing business models, we introduce two case studies from the water purification and metal degreasing fields, focusing on employees and local communities as two specific stakeholder groups of the company introducing Chemical Leasing. The paper seeks to demonstrate that Chemical Leasing business models can be branded as a corporate social responsibility initiative by outlining the vast potential of Chemical Leasing to improve occupational health and safety and to strengthen the ability of companies to protect the environment from the adverse effects of the chemicals they apply.     

Science with Society in the Anthropocene

Interdisciplinary scientific knowledge is necessary but not sufficient when it comes to addressing sustainable transformations, as science increasingly has to deal with normative and value-related issues. A systems perspective on coupled human–environmental systems (HES) helps to address the inherent complexities. Additionally, a thorough interaction between science and society (i.e., transdisciplinarity = TD) is necessary, as sustainable transitions are sometimes contested and can cause conflicts. In order to navigate complexities regarding the delicate interaction of scientific research with societal decisions these processes must proceed in a structured and functional way. We thus propose HES-based TD processes to provide a basis for reorganizing science in coming decades.     

Water quality perception,a dynamic evaluation

Seventy-six users of a camp grounds along the River Loing (France) took part in semi-directive interviews concerning their perceptions of water pollution levels of the river. The results of the interviews were analysed in conjunction with objective water pollution data supplied by the water quality monitoring authority. While there is some relationship between subjective estimates and objective quality classifications many people appear to be tolerant of water pollution. Quality estimates do not seem to be affected by the specific water-related activities of the respondents. A number of criteria are used by respondents in achieving their overall assessment of water quality (colour, presence/absence of water plants and algae, presence/absence of floating debris, odour, movement and clarity). These criteria appear to be used hierarchically and in a multi-stage judgmental process.     

Clearance of PCDD/Fs via the gastrointestinal tract in occupationally exposed persons.

A digestive tract mass balance was performed on six men with high body burdens of polychlorinated dibenzo-p-dioxins and dibenzofurans (PCDD/Fs). Intake via food was measured by analyzing duplicate portions of the food consumed by the volunteers and excretion via feces was determined by quantitative collection and analysis of the feces. Blood samples were taken to determine the current body burden. The results showed that the quantity of non-metabolized chemical excreted in the feces clearly exceeded the uptake via food for all of the 2,3,7,8-substituted PCDDs and some of the PCDFs, indicating a significant clearance across the gastrointestinal tract. The concentrations of these PCDD/F congeners in blood and feces were highly correlated (r > 0.8), demonstrating that the fecal PCDD/F content was determined by the body burden. The half lives in the test persons due to fecal clearance of non-metabolized chemical were estimated from the excretion rate and the current body burden and ranged between 10 years (Cl8DD) and 33 years (2,3,4,7,8-Cl5DF). These were compared with the overall contaminant half-lives due to all clearance processes which were calculated from the body burden and the decrease in blood concentrations measured over several years. The fecal clearance of non-metabolized PCDD/F contributed on average between 37% (2,3,7,8-Cl4DD) and 90% (Cl8DD) to the total elimination. This indicates that the gastrointestinal pathway plays a decisive role in the clearance of most 2,3,7,8-subsituted PCDD/F congeners.     

Partitioning of polychlorinated biphenyls and hexachlorobenzene into human faeces

The dietary absorption of persistent lipophilic organic pollutants (PLOPs) in humans is believed to occur via partitioning of the chemical between the lumen and the wall of the digestive tract. As such, the partitioning properties of the lumen contents are a key factor governing absorption. In this study, the partitioning properties of faeces were measured for 11 polychlorinated biphenyls and hexachlorobenzene (HCB). Four volunteers participated in the study, each of them providing faeces from a normal diet and a vegetarian diet. The faeces/gas equilibrium partition coefficient K(FG) varied by over three orders of magnitude between the different compounds. A linear relationship between log K(FG) and log KOA, the octanol/air partition coefficient, was observed. The slope of the relationship was > 1, indicating that the solvent properties of faeces were less polar than those of octanol. For a given compound, KFG varied up to a factor of 2.8 between the individuals on a normal diet. The influence of the vegetarian diet on K(FG) was negligible for the two volunteers who simply deleted fish and animal products from their normal diet, but K(FG) increased on average by a factor of 2 in the two individuals who increased their consumption of less readily digestible whole grains and vegetables in their vegetarian diet. On the basis of K(FG), the fugacities in the faeces were calculated. They were found to be much lower than the fugacities in blood. It is hypothesised that this is due to a temporary decrease in the fugacity in the wall of the jejunum caused by absorption of dietary lipids that results in equilibration between the lumen contents and the wall of the digestive tract at a fugacity below that present in the blood and the rest of the body.