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Neonatal hemochromatosis (NH) is a rare disorder but the most common cause of acute liver failure in neonates. NH is characterized by severe hepatic injury and iron overload and is associated with high perinatal mortality and morbidity rates. NH is often preceded by oligohydramnios and intrauterine growth restriction, suggesting an important impact of NH during fetal life. Stillbirth and prematurity are not uncommon. During the last decade, major discoveries on the etiology of NH have radically changed the management and outcome of this disease. NH is now regarded as an alloimmune disease and is, as such, often referred to as gestational alloimmune liver disease. Antenatal treatment with intravenous immunoglobulins starting at 14 weeks' gestation has been shown to prevent the development of NH in subsequent pregnancies. Postnatal treatment, previously based on the use of anti-oxidants and chelation therapy, has now successfully been replaced by exchange transfusions and intravenous immunoglobulins substitution. This review summarizes the latest discoveries on the etiology of NH and the new recommendations concerning its management and prevention. © 2013 John Wiley & Sons, Ltd.  相似文献   
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Accurate delineation and characterization of valley-bottom settings is crucial to the assessment of the biological and geomorphological components of riverine systems; yet, to date, most valley-bottom mapping endeavors have been done manually. To improve this situation, we developed automated techniques in a Geographic Information System (GIS) for delineating and characterizing valley-bottom settings in river basins ranging in size from approximately 1,000–10,000 km2. All procedures were developed with ARC/INFO GIS software and fully automated in Arc Macro Language (AML). The GRID module is required for valley-bottom delineation and slope calculations; whereas characterization (i.e., measuring the width of the valley-bottom zone) requires Coordinate Geometry (COGO) in the ARCEDIT module. The process requires three inputs: a polygon coverage of the analysis area; an arc coverage of its hydrography, and a grid representing its digital elevation. The AML is designed to operate within a wide range of computer memory/disk space options, and it allows users to customize several procedures to match the scale and complexity of a given analysis area with available computer hardware.  相似文献   
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Coastal catchments in British Columbia, Canada, experience a complex mixture of rainfall‐ and snowmelt‐driven contributions to flood events. Few operational flood‐forecast models are available in the region. Here, we integrated a number of proven technologies in a novel way to produce a super‐ensemble forecast system for the Englishman River, a flood‐prone stream on Vancouver Island. This three‐day‐ahead modeling system utilizes up to 42 numerical weather prediction model outputs from the North American Ensemble Forecast System, combined with six artificial neural network‐based streamflow models representing various slightly different system conceptualizations, all of which were trained exclusively on historical high‐flow data. As such, the system combines relatively low model development times and costs with the generation of fully probabilistic forecasts reflecting uncertainty in the simulation of both atmospheric and terrestrial hydrologic dynamics. Results from operational testing by British Columbia's flood forecasting agency during the 2013‐2014 storm season suggest that the prediction system is operationally useful and robust.  相似文献   
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As a result of climate changes, more land is now needed in the Netherlands for water retention in towns and the countryside. Recently, this perceived need has resulted in new forms of water management and spatial planning strategies. Multi-functional land use is an option such as housing in combination with retention lakes. Some argue that the market potential is high and that water has an added value in residential environments. This added value could be used to compensate for non-profitable investments such as the creation of lakes. The key question is how to value this amenity. It is argued that non-economists often assume that a valuation is just a technique. The authors challenge this by presenting an overview of economic perspectives as well as techniques. A literature review reveals the main valuation perspectives and suggests estimated added values of 10‐15% for the seashore, 5‐10% for river locations (streams), and 5% for lakes.  相似文献   
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The presence of glass-like contaminants inside waste glass products, usually resulting from both industrial and differentiated urban waste collection, has greatly increased in recent years, due to the introduction to the market of a large amount of goods manufactured from ceramic glass. The presence of contaminants in the glass recycling streams reduces product quality and increases production costs. The detection of ceramic glass detection is an unresolved problem, as such material looks like normal glass and can only be detected by trained personnel. In this study an innovative approach to ceramic glass recognition, based on the spectral signature in the mid-infrared (MIR) field, was proposed and investigated. The study specifically addressed the spectral characterization of glass and ceramic glass fragments collected in a real recycling plant from two different production lines: coloured container glass and white container glass. To define suitable inspection strategies to separate the useful (glass) from the polluting (ceramic glass) materials at the recycling plants, fragments presenting different colour, thickness, size, shape and manufacturing were selected. Both dirty and clean cullet was considered. The analyses, carried out in the MIR spectral field (2280-4480 nm), show that ceramic glass and glass fragments can be recognized according to their different spectral signature. In particular, by selecting a specific wavelength ratio the two classes of materials can be rapidly recognized, suggesting the possibility of developing an integrated hardware and software sorting system for 'on-line' ceramic glass separation.  相似文献   
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