Prenatal diagnosis of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in a family with a previous fatal case of sudden unexpected death in childhood

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is a potentially fatal inherited disease with a carrier frequency of approximately 1:100 in most Caucasian populations. The disease is implicated in sudden unexpected death in childhood. A prevalent disease-causing point mutation (A985G) in the MCAD gene has been characterized, thus rendering diagnosis easy in the majority of cases. Since the clinical spectrum of MCAD deficiency ranges from death in the first days of life to an asymptomatic life, there are probably other genetic factors—in addition to MCAD mutations—involved in the expression of the disease. Thus, families who have experienced the death of a child from MCAD deficiency might have an increased risk of a seriously affected subsequent child. In such a family we have therefore performed a prenatal diagnosis on a chorionic villus sample by a highly specific and sensitive polymerase chain reaction (PCR) assay for the G985 mutation. The analysis was positive and resulted in abortion. We verified the diagnosis by direct analysis on blood spots and other tissue material from the aborted fetus and from family members.     

A New Modus Operandi for the Agricultural Economics Profession

Agricultural economics has, until the 1990s, enjoyeda reputation for relevance and usefulness to theagri-food industry and policy-makers. Thatreputation has been jeopardized by a growinginfatuation with models and quantification, and aconcomitant underemphasis placed on many complexproblems and issues of society. An illustrativeexample is explored, using agriculturalactivity-related damage to the natural resourcebase, environment and ecology. Agriculturaleconomists are urged to respond by broadening theirterms of reference and joining forces with otherdisciplines.     

Modelling municipal solid waste generation: a review

The objective of this paper is to review previously published models of municipal solid waste generation and to propose an implementation guideline which will provide a compromise between information gain and cost-efficient model development. The 45 modelling approaches identified in a systematic literature review aim at explaining or estimating the present or future waste generation using economic, socio-demographic or management-orientated data. A classification was developed in order to categorise these highly heterogeneous models according to the following criteria--the regional scale, the modelled waste streams, the hypothesised independent variables and the modelling method. A procedural practice guideline was derived from a discussion of the underlying models in order to propose beneficial design options concerning regional sampling (i.e., number and size of observed areas), waste stream definition and investigation, selection of independent variables and model validation procedures. The practical application of the findings was demonstrated with two case studies performed on different regional scales, i.e., on a household and on a city level. The findings of this review are finally summarised in the form of a relevance tree for methodology selection.     

An Approach for Evaluating the Effectiveness of Various Ozone Air Quality Standards for Protecting Trees

We demonstrate an approach for evaluating the level of protection attained using a variety of forms and levels of past, current, and proposed Air Quality Standards (AQSs). The U.S. Clean Air Act requires the establishment of ambient air quality standards to protect health and public welfare. However, determination of attainment of these standards is based on ambient pollutant concentrations rather than prevention of adverse effects. To determine if a given AQS protected against adverse effects on vegetation, hourly ozone concentrations were adjusted to create exposure levels that “just attain” a given standard. These exposures were used in combination with a physiologically-based tree growth model to account for the interactions of climate and ozone. In the evaluation, we used ozone concentrations from two 6-year time periods from the San Bernardino Mountains in California. There were clear differences in the level of vegetation protection achieved with the various AQSs. Based on modeled plant growth, the most effective standards were the California 8-hr average maximum of 70 ppb and a seasonal, cumulative, concentration-weighted index (SUM06), which if attained, resulted in annual growth reductions of 1% or less. Least effective was the 1-hr maximum of 120 ppb which resulted in a 7% annual reduction. We conclude that combining climate, exposure scenarios, and a process-based plant growth simulator was a useful approach for evaluating effectiveness of current or proposed air quality standards, or evaluating the form and/or level of a standard based on preventing adverse growth effects.     

Multiple song repertoire characteristics in the willow warbler (Phylloscopus trochilus): correlations with female choice and offspring viability

Male willow warblers have song repertoires which vary in complexity along several dimensions. We examined whether female choice, as measured by date of pairing, was based on these song characteristics in 4 different years. Pairing date was negatively correlated with song repertoire size in 1 year, and with song versatility in another year, but there was no consistent effect of any song characteristic on pairing throughout the years or in the pooled sample. The variable that best explained how soon a male pairs is male arrival date (only males that had settled territories before the first female arrived were considered in the analysis). This correlation is consistently significant in all years. This is most parsimoniously interpreted as females choosing some habitat characteristic in the same way that males do. A small percentage of males (8.3%) attracted and paired with a second female. The likelihood of becoming polygynous was not explained by any measured song characteristic, but it was related to arrival date: early males were more likely to pair with two females. Males with large repertoires fledged more young in their primary nests, and there was a trend for the offspring of these males to have a greater probability of being recruited into the population. In conclusion, the results show that in most years there is no sexual selection by female preference on song characteristics, although the data on reproductive success is consistent with the idea of repertoire size being an indicator of male quality. Received: 4 June 1999 / Received in revised form: 1 December 1999 / Accepted: 31 December 1999