Cerebro-costo-mandibular syndrome in a father and a female fetus: early prenatal ultrasonographic diagnosis and autosomal dominant transmission

Ultrasonography in a female fetus revealed cystic cervical hygroma, severe micrognathia, and vertebral and upper limb anomalies suggestive of cerebro-costo-mandibular syndrome (CCMS) which was diagnosed ultrasonographically at 16 weeks' gestation. The father is affected and presents with a Pierre Robin sequence, short stature and typical costovertebral anomalies. CCMS is a rare and severe disorder. The high frequency of sporadic cases, vertical transmission, and the excess of sibs affected via horizontal transmission suggest dominant autosomal mutation with possible germinal mosaicism. The vertical familial case detailed in the present report is a reminder of the high risk when one parent or one sibling is affected and the extreme variability of phenotype and costal ossification. Early prenatal ultrasound diagnosis is possible in a severely affected fetus. Copyright © 2001 John Wiley & Sons, Ltd.     

Validation of FLACS against experimental data sets from the model evaluation database for LNG vapor dispersion

The siting of facilities handling liquefied natural gas (LNG), whether for liquefaction, storage or regasification purposes, requires the hazards from potential releases to be evaluated. One of the consequences of an LNG release is the creation of a flammable vapor cloud, that may be pushed beyond the facility boundaries by the wind and thus present a hazard to the public. Therefore, numerical models are required to determine the footprint that may be covered by a flammable vapor cloud as a result of an LNG release. Several new models have been used in recent years for this type of simulations. This prompted the development of the “Model evaluation protocol for LNG vapor dispersion models” (MEP): a procedure aimed at evaluating quantitatively the ability of a model to accurately predict the dispersion of an LNG vapor cloud.This paper summarizes the MEP requirements and presents the results obtained from the application of the MEP to a computational fluid dynamics (CFD) model – FLACS. The entire set of 33 experiments included in the model validation database were simulated using FLACS. The simulation results are reported and compared with the experimental data. A set of statistical performance measures are calculated based on the FLACS simulation results and compared with the acceptability criteria established in the MEP. The results of the evaluation demonstrate that FLACS can be considered a suitable model to accurately simulate the dispersion of vapor from an LNG release.     

Modeling organizational processes across hierarchical levels: climate,leadership, and group process in work groups

Perceptions of organizational climate, leadership, and group processes were aggregated within hierarchically nested work groups. Relationships across hierarchical boundaries were examined for two samples at different hierarchical levels in a military organization. Perceptions of climate were positively related across levels in both samples. There was evidence that the pattern of relationship among the other constructs was different in the two samples. The results have implications for the process which organizational interventions can be expected to flow through hierarchical levels of an organization. © 1997 John Wiley & Sons, Ltd.     

A framework for testing meso‐mediational relationships in Organizational Behavior

We advance a framework for testing mediational relationships that traverse levels of analysis. We differentiate mediation that functions within levels, as well as different forms of meso‐mediation that operate downward across levels of analysis. We discuss the preconditions and evidential basis for these meso‐mediational type relationships. We then provide an empirical example that illustrates the different types of mediation and conclude with recommendations and extensions for studying more complex meso‐mediational organizational phenomena. Copyright © 2007 John Wiley & Sons, Ltd.     

Dynamics of copper and zinc sedimentation in a lagooning system receiving landfill leachate

This study characterises the sediment dredged from a lagooning system composed of a settling pond and three lagoons that receive leachates from a municipal solid waste (MSW) landfill in France. Organic carbon, carbonate, iron oxyhydroxides, copper (Cu) and zinc (Zn) concentrations were measured in the sediment collected from upstream to downstream in the lagooning system. In order to complete our investigation of sedimentation mechanisms, leachates were sampled in both dry (spring) and wet (winter) seasonal conditions. Precipitation of calcite and amorphous Fe-oxyhydroxides and sedimentation of organic matter occurred in the settling pond. Since different distributions of Zn and Cu concentrations are measured in sediment samples collected downstream in the lagooning system, it is suggested that these elements were not distributed in a similar way in the leachate fractions during the first stage of treatment in the settling pond, so that their sedimentation dynamics in the lagooning system differ. In the lagoons, it was found that organic carbon plays a major role in Cu and Zn mobility and trapping. The presence of macrophytes along the edges provided an input of organic matter that enhanced Cu and Zn scavenging. This edge effect resulted in a two-fold increase in Cu and Zn concentrations in the sediment deposited near the banks of the lagoons, thus confirming the importance of vegetation for the retention of Cu and Zn in lagooning systems.     

Quantifying the contribution of nitrification and denitrification to the nitrous oxide flux using 15N tracers

Microbial transformations of nitrification and denitrification are the main sources of nitrous oxide (N2O) from soils. Relative contributions of both processes to N2O emissions were estimated on an agricultural soil using 15N isotope tracers (15NH4+ or 15NO3-), for a 10-day batch experiment. Under unsaturated and saturated conditions, both processes were significantly involved in N2O production. Under unsaturated conditions, 60% of N-N2O came from nitrification, while denitrification contributed around 85-90% under saturated conditions. Estimated nitrification rates were not significantly different whatever the soil moisture content, whereas the proportion of nitrified N emitted as N2O changed from 0.13 to 2.32%. In coherence with previous studies, we interpreted this high value as resulting from the decrease in O2 availability through the increase in soil moisture content. It thus appears that, under limiting aeration conditions, some values for N2O emissions through nitrification could be underestimated.     

On the wind resource mapping of Burkina Faso

In this work, mesoscale wind resource maps, at 5-km resolution, of the country of Burkina Faso (274,200 km²) were developed using the Anemoscope and mesoscale compressible community models. Results show that the northeast region of Burkina Faso has a good wind regime at 80 m above ground level (agl), while the wind regime in other parts of the country is generally low, even at 80 m agl. In addition, the technical power potential and the potential annual energy production that can be generated from the wind in Burkina Faso are identified using analysis tools based on geographical information systems and economic constraints. Results from the technical power potential at 80 m agl show that a total of 312 MW of wind farms, generating annually a total of 741 GWh of energy, could be installed in Burkina Faso. On the other hand, a total of 4411 MW of small wind turbines (50 kW) could be installed over the territory, corresponding to an annual energy production of 7843 GWh. The Wind Atlas of Burkina Faso provides an opportunity for local stakeholders to consider wind energy for the electricity portfolio of the country.     

Clarifying conditions and decision points for mediational type inferences in Organizational Behavior

Although mediational designs and analyses are quite popular in Organizational Behavior research, there is much confusion surrounding the basis of causal inferences. We review theoretical, research design, and construct validity issues that are important for drawing inferences from mediational analyses. We then distinguish between indirect effects, and partial and full mediational hypotheses and outline decision points for drawing inferences of each type. An empirical illustration is provided using structural equation modeling (SEM) techniques, and we discuss extensions and directions for future research. Copyright © 2006 John Wiley & Sons, Ltd.