Split-hand/split-foot malformation with paternal mutation in the p63 gene

We report the prenatal diagnosis at 16 weeks' gestation of bilateral split-hand/split-foot malformation (SHSFM) with severe lobster claw deformity of hands and feet in a male fetus without associated malformations. A minor manifestation of SHSFM was present in the father with only mild bilateral foot involvement (syndactyly I–II; cleft II–III; left cutaneous syndactyly III–IV). Mutation analysis of the p63 gene on chromosome 3q27 showed a missense mutation 577A→G (predicting amino acid substitution K193E) in the father. This mutation has not been reported so far in SHSFM but resembles the previously reported 580A→G (predicting amino acid substitution K194E) in a family with SHSFM. Copyright © 2001 John Wiley & Sons, Ltd.     

Direct measurements of the drag force over aligned arrays of cubes exposed to boundary-layer flows

Wind tunnel measurements of the total drag force for aligned arrays of cubes exposed to two different boundary-layer flows at three flow velocities are discussed. The drag force for eight different building packing densities λ _p (from 0.028 to 1) is measured with a standard load cell generating a novel dataset. Different λ _p are reproduced by increasing the number of buildings on the same lot area; this represents a real situation that an urban planner is faced with when a lot area of a given (fixed) size is allocated to the development of new built areas. It is assumed that the surrounding terrain is uniform and there is a transition from a given roughness (smooth) to a new roughness (rough). The approaching flow will adjust itself over the new surface within a distance that in general may be larger than the horizontal length covered by the array. We investigate the region where the flow adjustment occurs. The wide range of packing densities allowed us to analyse in detail the evolution of the drag force. The drag force increases with increasing packing densities until it reaches a maximum at an intermediate packing density (λ _p  = 0.25 in our case) followed by a slight decrease at larger packing densities. The value of the drag force depends on the flow adjustment along the array which is evaluated by introducing the parameter “drag area” to retrieve information about the drag distribution at different λ _p . Results clearly suggest a change of the distribution of the drag force, which is found to be relatively uniform at low packing densities, while most of the force acts on first rows of the arrays at large packing densities. The drag area constitutes the basis for the formulation of a new adjustment length scale defined as the ratio between the volume of the air within the array and the drag area. The proposed adjustment length scale automatically takes into account the change in drag distribution along the array for a better parameterization of urban effects in dispersion models.     

Aerosol chemical characteristics of an island site in the Bay of Bengal (Bhola-Bangladesh)

Aerosol constituents (elemental carbon, organic carbon, soluble ions including organic acids, and selected trace metals) were investigated from samples of a field campaign taking place at Bhola Island in the Bay of Bengal (Bangladesh). The campaign took place in the pre-monsoon season (May 2001) using low volume samplers. Carbonaceous material comprised the majority of the analysed components. The average concentrations of EC and OC were 2.8 and 4.6 microg m(-3), respectively. Oxalic acid was the most abundant dicarboxylic acid (average 268 ng m(-3)) followed by malonic and malic acid. The contribution of carboxylic acids-carbon to organic carbon was 2.0%. Average concentrations observed for sulfate and nitrate were 3.7 microg m(-3) and 1.5 microg m(-3). Two different types of aerosol were identified at the rural background site on Bhola Island during southerly synoptic flow by means of trajectory analysis: air masses were transported from the Bay of Bengal to the sampling site in all cases. However, during "Period 1" they experienced longer residence times over the Indian Ocean, while the "Period 2" trajectories came along the Indian coast or passed over the Indian continent. During Period 1 the concentration levels of soluble ions were a factor of 4-6 lower than during Period 2. The concentrations of EC, OC and K differed less than a factor of 1.5 between the two periods. The Period 1 aerosol showed similarities to the haze layers observed during winter-monsoon conditions south of India during the INDOEX experiment. Based on EC/TC and K/EC ratios we find that around 80% of the carbonaceous aerosol from Period 1 in Bhola is from fossil fuel and only around 50% from Period 2. Absolute concentrations of carbonaceous species, soluble ions and trace metals indicate that the background site on Bhola Island is affected by emissions from urbanized regions of Southeast Asia.     

Risk measures constituting a risk metrics which enables improved decision making: Value-at-Risk

In this work, a novel approach is proposed for expressing the risks of process plants consisting of a large number of scenarios, in the form of a risk metrics of leading indicators to prevent potential high profile industry accidents. The methodology includes: 1) risk estimation of a portfolio by CPQRA (or QRA), 2) monetization of the tangible risks with the inclusion of the lost time of production, 3) estimation of the maximum portfolio loss using Value-at-Risk approach, 4) inclusion of intangible risks using FN-curve and, 5) generation of F$-curve of tangible risks. The proposed methodology can particularly help in understanding the stakes at risk by performing the overall cost-benefit analysis, for identifying the most risky scenarios and identifying critical equipments to enable better risk-informed decision making in order to adopt appropriate risk mitigation measures. This work establishes the groundwork for developing measures for understanding and comparing the large number of risk values derived from QRA studies for large portfolios. It will aid in less subjective decision making as it enables the decision maker to choose the most preferred portfolio option among alternatives. Decisions made with the accurate understanding of the consequences of risks can significantly reduce potential work-related fatalities, property losses and save millions of dollars.     

Safety challenges in view of the upcoming hydrogen economy: An overview

If produced and arranged in the right way with carbon dioxide as a possible but permanently removed by-product, hydrogen as a new, large scale, applicable energy carrier promises significant reductions of carbon dioxide emissions. It is light, non-toxic, and clean burning. In different parts of the world, viz. United States, Japan, and Europe, programs have started some years ago to investigate hazardous properties of hydrogen in more detail and to develop special safety measures where necessary. Recently, in September 2009, the third International Conference on Hydrogen Safety (3rd ICHS) was held at Corsica, France. CFD tools have been adapted to describe hydrogen dispersion and explosion. Field tests have been carried out, an incident databank has been founded, knowledge gaps were defined, and risk analysis methods reviewed. The latter are required for drafting installation guidelines and measures for safe distances in land-use planning and licensing of hydrogen storage locations, pipelines, and re-fuelling stations. Yet some challenges remain. The paper will summarize present results and will address hydrogen related issues where more knowledge is needed to reduce uncertainty and improve the quality of risk control.