Prenatal ultrasound diagnosis of Toriello–Carey syndrome

Toriello–Carey syndrome is a rare malformative complex, described for the first time in 1988, characterized by agenesis of the corpus callosum, facial anomalies, cardiac defects and hypotonia. Relatively few neonatal cases have been reported. We describe here the first prenatal ultrasound diagnosis of the syndrome based on the detection of agenesis of the corpus callosum and spongious cardiomyopathy in a 22-week-old fetus of a couple with positive family history. The first sib of the couple was diagnosed with Toriello–Carey syndrome at 1 year of age, and had, in addition to the typical facial anomalies not detectable by ultrasound, agenesis of the corpus callosum and the same heart lesion (spongious cardiomyopathy). This report demonstrates that prenatal diagnosis of Toriello–Carey syndrome is feasible in the second trimester of pregnancy. Copyright © 2002 John Wiley & Sons, Ltd.     

Foreword

Mitigation and Adaptation Strategies for Global Change -     

Ecosystem management to achieve ecological sustainability: The case of South Florida

The ecosystems of South Florida are unique in the world. The defining features of the natural Everglades (large spatial scale, temporal patterns of water storage and sheetflow, and low nutrient levels) historically allowed a mosaic of habitats with characteristic animals. Massive hydrological alterations have halved the Everglades, and ecological sustainability requires fundamental changes in management.The US Man and the Biosphere Human-Dominated Systems Directorate is conducting a case study of South Florida using ecosystem management as a framework for exploring options for mutually dependent sustainability of society and the environment. A new methodology was developed to specify sustainability goals, characterize human factors affecting the ecosystem, and conduct scenario/consequence analyses to examine ecological and societal implications. South Florida has sufficient water for urban, agricultural, and ecological needs, but most water drains to the sea through the system of canals; thus, the issue is not competition for resources but storage and management of water. The goal is to reestablish the natural system for water quantity, timing, and distribution over a sufficient area to restore the essence of the Everglades.The societal sustainability in the Everglades Agricultural Area (EAA) is at risk because of soil degradation, vulnerability of sugar price supports, policies affecting Cuban sugar imports, and political/economic forces aligned against sugar production. One scenario suggested using the EAA for water storage while under private sugar production, thereby linking sustainability of the ecological system with societal sustainability. Further analyses are needed, but the US MAB project suggests achieving ecological sustainability consistent with societal sustainability may be feasible.     

Development of Layered Sediment Structure and its Effects on Pore Water Transport and Hyporheic Exchange

Hyporheic exchange is known to provide an important control on nutrient and contaminant fluxes across the stream-subsurface interface. Similar processes also mediate interfacial transport in other permeable sediments. Recent research has focused on understanding the mechanics of these exchange processes and improving estimation of exchange rates in natural systems. While the structure of sediment beds obviously influences pore water flow rates and patterns, little is known about the interplay of typical sedimentary structures, hyporheic exchange, and other transport processes in fluvial/alluvial sediments. Here we discuss several processes that contribute to local-scale sediment heterogeneity and present results that illustrate the interaction of overlying flow conditions, the development of sediment structure, pore water transport, and stream-subsurface exchange. Layered structures are shown to develop at several scales within sediment beds. Surface sampling is used to analyze the development of an armor layer in a sand-and-gravel bed, while innovative synchrotron-based X-ray microtomography is used to observe patterns of grain sorting within sand bedforms. We show that layered bed structures involving coarsening of the bed surface increase interfacial solute flux but produce an effective anisotropy that favors horizontal pore water transport while limiting vertical penetration.     

The potential for improvements in energy efficiency and CO2 emissions in the EU27 cement industry and the relationship with the capital budgeting decision criteria

This paper performs a cost-effectiveness analysis of some of the best available technologies (BAT) that can contribute to decreasing the energy consumption and CO₂ emissions in the European Union’s (EU27) cement industry. Several capital budgeting decision criteria have been used (the payback period, the net present value and the internal rate of return) to study whether or not an investment should be considered worthwhile. The results show that, independent of the capital budgeting decision criteria used, the number of cost-effective retrofitting possibilities available is large compared to the rate of improvements that the industry undertakes annually. This shows the insensitivity of the industry to financial criteria when it comes to making their investment decisions. The possible thermal energy improvement in the clinker production, if all these BATs were implemented, has been quantified to be around 10%. This achievement would place the cement industry in the upper bound of the benchmark range for clinker manufacture. However considering the delays observed nowadays in terms of diffusion of BATs in the cement industry, it requires a conducive policy environment that combines support for both technology development and to their deployment.     

Innovation for sustainable development in artisanal mining: Advances in a cluster of opal mining in Brazil

The objective of this article is to understand how the promotion of clusters of small economic agents in the gem sector has brought some improvements in the economic, social and environmental conditions in the mining sector and activities related to it. The research provides policy and theoretical contributions to the field of gem production, as well as enhances understanding of the under researched opal production in Brazil. It argues that government funding and technical support dedicated to the development of mining clusters, i.e. working with small economic agents as a whole and not individually, could promote not only more economic development, but also effectively incorporate social and environmental issues, such as workers safety, water management and tailings recycling. The argument is based on an evaluation of environmental, economic, social and institutional aspects of the opal mining cluster in Pedro II municipality, Piauí state. The results suggest that some formalisation of existing practices and adequate policies have triggered innovation with some positive effects on the performance of artisanal mining. Nevertheless, there are indications that if decision-makers plan to make this cluster more sustainable, they will have to include other issues in the debate including developing substituting economic activities.     

Effects of Water Availability and Habitat Quality on Bark-Stripping Behavior in Barbary Macaques

Abstract: The cedar oak forest of the Middle Atlas in Morocco is not only the last of the large forests in the southern Mediterranean, but it also contains all the surviving forest biodiversity. This forest has been severely affected by drought, overgrazing by mixed herds of goat and sheep, and excessive logging for timber, firewood, and livestock fodder. Recently, cedar bark stripping by Barbary Macaques (    Macaca sylvanus ) has begun to have an effect on the forest. We investigated this behavior by monitoring a 500-km² mosaic forest of cedar and oak in the Middle Atlas of Morocco between 1994 and 1996. We surveyed the forest 18 times in four different seasons along a 90-km transect. We recorded observations of bark stripping and a variety of quantitative ecological factors that could predict this behavior, such as livestock density, forest quality, undergrowth condition, water availability, and monkey density. The statistical analysis (including rank correlation, regression, and nonparametric variance analysis) strongly suggests that water scarcity and monkey exclusion from previously available permanent water sources are correlated with intense cedar bark-stripping behavior by macaques. The density of cedars and of monkeys appeared to be only secondary factors. As a conservation policy, making water more accessible to wild monkeys might reduce bark-stripping behavior.     

Energy efficacy used to score organic refuse pretreatment processes for hydrogen anaerobic production

The production of hydrogen through Anaerobic Digestion (AD) has been investigated to verify the efficacy of several pretreatment processes. Three types of waste with different carbon structures have been tested to obtain an extensive representation of the behavior of the materials present in Organic Waste (OW). The following types of waste were selected: Sweet Product Residue (SPR), i.e., confectionary residue removed from the market after the expiration date, Organic Waste Market (OWM) refuse from a local fruit and vegetable market, and Coffee Seed Skin (CSS) waste from a coffee production plant. Several pretreatment processes have been applied, including physical, chemical, thermal, and ultrasonic processes and a combination of these processes. Two methods have been used for the SPR to remove the packaging, manual (SPR) and mechanical (SPRex). A pilot plant that is able to extrude the refuse to 200 atm was utilized. Two parameters have been used to score the different pretreatment processes: efficiency (ξ), which takes into account the amount of energy produced in the form of hydrogen compared with the available energy embedded in the refuse, and efficacy (η), which compares the efficiency obtained using the pretreated refuse with that obtained using the untreated refuse. The best result obtained for the SPR was the basic pretreatment, with η = 6.4, whereas the thermal basic pretreatment gave the highest value, η = 17.0 for SPRex. The best result for the OWM was obtained through a combination of basic/thermal pretreatments with η = 9.9; lastly, the CSS residue with ultrasonic pretreatment produced the highest quantity of hydrogen, η = 5.2.