A new report of mesomelic camptomelia,polydactyly and Dandy–Walker complex in siblings

Two male siblings with several malformations are reported. The anomalies detected in both fetuses were mesomelic camptomelia, postaxial hexadactyly and Dandy–Walker complex. There was only one similar previous report in the literature. This combination could represent a specific pattern of malformation or a new syndrome, with different variants. The parents' consanguinity and the recurrence in a subsequent pregnancy suggest an autosomal recessive inheritance pattern. Copyright © 2003 John Wiley & Sons, Ltd.     

Rational pricing of groundwater with a growing population

When population is increasing, characterizing the optimal water consumption path is complicated by the fact that the underlying dynamics of the water stock is contingent on the level of the stock itself. We propose a method of constructing the optimal path in this case. Since population is increasing, the optimal consumption path may involve refraining at times from consuming the totality of the surface water flow in order to restock in groundwater for future consumption. The aquifer then serves as a means to achieve welfare increasing intertemporal transfers of surface water. Therefore the aquifer itself, as distinct from the stock of water it serves to store, may have value and the marginal valuation of water when groundwater stocks are being drawn upon should, for this reason, differ at times from the marginal valuation of water when it is drawn strictly from surface water.     

A side-by-side comparison of two systems of sequencing coupled reactors for anaerobic digestion of the organic fraction of municipal solid waste.

The objective of this work was to compare the performance of two laboratory-scale, mesophilic systems aiming at the anaerobic digestion of the organic fraction of municipal solid wastes (OFMSW). The first system consisted of two coupled reactors packed with OFMSW (PBR1.1-PBR1.2) and the second system consisted of an upflow anaerobic sludge bed reactor (UASB) coupled to a packed reactor (UASB2.1-PBR2.2). For the start-up phase, both reactors PBR 1.1 and the UASB 2.1 (also called leading reactors) were inoculated with a mixture of non-anaerobic inocula and worked with leachate and effluent full recirculation, respectively. Once a full methanogenic regime was achieved in the leading reactors, their effluents were fed to the fresh-packed reactors PBR1.2 and PBR2.2, respectively. The leading PBR 1.1 reached its full methanogenic regime after 118 days (Tm, time to achieve methanogenesis) whereas the other leading UASB 2.1 reactor reached its full methanogenesis regime after only 34 days. After coupling the leading reactors to the corresponding packed reactors, it was found that both coupled anaerobic systems showed similar performances regarding the degradation of the OFMSW. Removal efficiencies of volatile solids and cellulose and the methane pseudo-yield were 85.95%, 80.88% and 0.109 NL CH4 g(-1) VS(fed) in the PBR-PBR system; and 88.75%, 82.61% and 0.115 NL CH4 g(-1) VS(fed0 in the UASB-PBR system [NL, normalized litre (273 degrees K, 1 ata basis)]. Yet, the second system UASB-PBR system showed a faster overall start-up.     

Environmental equity and electric power generation: Disparate community outcomes within Texas?

The examinations conducted in this study focus at the community level to test for disparate outcomes involving utility-based electric power generation within the crucial state of Texas. Potential policy implications are discussed as relevant to the general thesis of environmental racism postulated by justice advocates and the civil rights strategies advanced by justice advocates. Cross-sectional and longitudinal perspectives are used in testing for distributive inequities involving locations of fossil fuel power plants and emissions and output rates of emissions originating from such power plants. These tests provide empirical basis for assessing outcomes of the siting and emissions regulatory processes. While the civil rights strategies that would use Title VI of the Civil Rights Act of 1964 and the disparate impact standard are inapplicable, some limited findings indicate disparate outcomes involving other disadvantaged populations that are difficult to justify in context of legitimate market dynamics. Issues raised in this study have relevance to national energy policy proposals that promote many more power plants across the USA and encourage emissions trading.     

Mitochondrial DNA Variability in the Endangered Razorback Sucker (Xyrauchen texanus): Analysis of Hatchery Stocks and Implications for Captive Propagation

The razorback sucker ( Xyrauchen texanus ) is a large, long-lived catostomid fish endemic to the Colorado River drainage of western North America, endangered because of recruitment failure. Efforts to preserve the species have emphasized artificial propagation and reintroduction. Given the importance of maintaining genetic diversity in such a program, we examined mitochondrial DNA diversity in a source population (Lake Mohave, Arizona-Nevada) and three hatchery-produced year classes (1987, 1989, 1990). The source contained considerable variation, indicated by high haplotype diversity ( ĥ = 0.97) and a large number of unique haplotypes (17 in 25 individuals). Diversity also was high in the 1987 ( ĥ = 0.89, 6 haplotypes in 10 individuals) and 1989 hatchery-produced year classes ( ĥ = 0.91, 7 in 11), but significantly lower in the 1990 year class ( ĥ = 0.71, 4 in 10). Low diversity in the last class was likely because of differences among females in fecundity, viability of progeny, or both. Because natural populations have collapsed throughout the species' range, we must identify methods that preserve the most diversity. We examined three potential alternatives: standard hatchery propagation, natural spawning in predator-free environments, and protective custody of larvae collected from the lake with reintroduction after growth to a size likely to survive. The last is the preferred alternative and should be pursued as the most cost-effective option for preserving genetic diversity in the razorback sucker.     

Duplex PCR for preimplantation genetic diagnosis (PGD) of spinal muscular atrophy

The main difficulty in developing a molecular diagnosis of spinal muscular atrophy (SMA) resides in the specific genomic structure of the locus. Indeed, two highly homologous survival motor neurone genes, SMN1 and SMN2, are present at the locus. The detection of the homozygous deletion of exons 7 and 8 of the SMN1 gene, which is present in 90 to 98% of the patients, is based on methods highlighting 1 of the 8 nucleotidic mismatches existing between these 2 genes. In order to offer preimplantation genetic diagnosis (PGD) for SMA, we developed a new allele-specific amplification method. The main disadvantage of our previously described strategy resided in the possibility of diagnosing, in case of amplification failure, an unaffected embryo as affected. We present here a new PGD-SMA method. We established the conditions for three different duplex PCRs, allowing the specific detection of the SMN1 gene and one polymorphic marker, either D5S629, D5S1977, or D5S641. Of the 60 to 90 single cells tested, the PCR efficiency varied from 98 to 100% with a complete genotype obtained in a range between 81 and 87% with a global allele drop-out rate of 9%. Such a test was used to perform 1 PGD cycle for which 7 embryos could be analysed. All the embryos were fully diagnosed, six as unaffected and one as affected. Four embryos were transferred, but no pregnancy ensued. Copyright © 2003 John Wiley & Sons, Ltd.     

Advisory Committee: A Powerful Tool for Helping Decision Makers in Environmental Issues

/ It has been suggested that the general public should be moreinvolved in environmental policy and decision making. It is important forthem to realize that they will have to live with the consequences ofenvironmental policies and decisions. Consequently, policy makers shouldconsider the concerns and opinions of the general public before makingdecisions on environmental issues. This raises questions such as: How can weintegrate the perceptions and reactions of the general population inenvironmental decisions? What kind of public participation should weconsider? In the present study, using a new regional ecosystem model, weattempted to integrate these aspects in its decision making model byincluding the formation of an advisory committee to resolve problems relatedto waste management. The advisory committee requested the activeparticipation of representatives from all levels of the community: economic,municipal, and governmental intervenors; environmental groups; and citizens.Their mandates were to examine different management strategies available inthe region, considering all the interdisciplinary aspects of each strategy,elaborate recommendations concerning the management strategies that are mostsuitable for all, and collaborate in communication of the information to thegeneral population. The results showed that at least in small municipalitiessuch an advisory committee can be a powerful tool in environmental decisionmaking. Conditions required for a successful consultation process, such aseveryday lay language and the presence of a facilitator other than ascientific expert, are discussed.KEY WORDS: Public consultation; Environmental policies;Interdisciplinary aspects; Municipal sewage sludge management; Generalpopulation; Decision-making process     

Environmental performance evaluation of agro-industrial innovations – Part 2: methodological approach for performing vulnerability analysis of watersheds

Environmental vulnerability analysis has been sparsely used in environmental performance evaluation (EPE) of technological innovations. The present paper proposes a methodological approach to carry out vulnerability analysis of watersheds and to integrate this analysis into methods of environmental performance evaluation of agro-industrial innovations. This approach is applied to the Ambitec-Life Cycle method, described in Part 1 (this issue) of this study. The case study of green coconut substrate compared to ripe coconut substrate, also described in Part 1 (this issue), is now presented considering the vulnerability analysis of the watersheds where the life cycle stages of these products occur. The integration of vulnerability analysis in Ambitec-Life Cycle contributes to a better understanding of the environmental aspects of agro-industrial technological innovations with potential to cause significant impacts in watersheds where these innovations are implemented.