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121.
Pfeiffer syndrome is an autosomal dominant disorder characterized by coronal craniosynostosis, midface hypoplasia, broad thumbs and great toes. On the basis of clinical findings, three subtypes have been delineated. The clinical variability of Pfeiffer syndrome as well as other causes of craniosynostosis can make a prenatal diagnosis based on sonography alone difficult. We describe a fetus in whom sonographic findings (including 3D ultrasound) suggested a Pfeiffer syndrome type II and in which subsequent molecular analysis verified the diagnosis by identifying a de novo mutation in the FGFR2 gene. To the best of our knowledge, this is the first report of a prenatal molecular diagnosis of Pfeiffer syndrome in a patient without family history. Copyright © 2004 John Wiley & Sons, Ltd.  相似文献   
122.
An encapsulation system designed to give long-lasting effectiveness of the insect growth regulator (IGR) cyromazine (Neporex) against Culex pipiens (Culicidae) mosquito larvae was developed. The main thrust of the research was to develop a simple method for preparing controlled-release formulations by means of an extrusion technique in combination with a supplementary coating and to study the release characteristics and effectiveness of the formulations. A series of formulations were prepared with low-density polyethylene (LDPE 600) for the matrix and with polyurea or one of four types of polyurethane for the coating. The rate of release of the active material from the controlled-release formulations was determined in an in vitro dissolution system. The biological activity of the controlled-release formulations was tested in vivo against C. pipiens larvae.  相似文献   
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Using the GAINS (Greenhouse Gas–Air Pollution Interactions and Synergies) model, we derived Annex I marginal abatement cost curves for the years 2020 and 2030 for three World Energy Outlook baseline scenarios (2007–2009) of the International Energy Agency. These cost curves are presented by country, by greenhouse gas and by sector. They are available for further inter-country comparisons in the GAINS Mitigation Efforts Calculator—a free online tool. We illustrate the influence of the baseline scenario on the shape of mitigation cost curves, and identify key low cost options as well as no-regret priority investment areas for the years 2010–2030. Finally, we show the co-effect of GHG mitigation on the emissions of local air pollutants and argue that these co-benefits offer strong local incentives for mitigation.  相似文献   
125.
This paper provides an analysis of co-benefits for traditional air pollutants made possible through global climate policies using the Greenhouse Gas and Air Pollution Interactions and Synergies (GAINS) model in the time horizon up to 2050. The impact analysis is based on projections of energy consumption provided by the Prospective Outlook for the Long term Energy System (POLES) model for a scenario without any global greenhouse gas mitigation efforts, and for a 2°C climate policy scenario which assumes internationally coordinated action to mitigate climate change. Outcomes of the analysis are reported globally and for key world regions: the European Union (EU), China, India and the United States. The assessment takes into account current air pollution control legislation in each country. Expenditures on air pollution control under the global climate mitigation regime are reduced in 2050 by 250 billion € when compared to the case without climate measures. Around one third of financial co-benefits estimated world-wide in this study by 2050 occur in China, while an annual cost saving of 35 billion (Euros) € is estimated for the EU if the current air pollution legislation and climate policies are adopted in parallel. Health impacts of air pollution are quantified in terms of loss of life expectancy related to the exposure from anthropogenic emissions of fine particles, as well as in terms of premature mortality due to ground-level ozone. For example in China, current ambient concentrations of particulate matter are responsible for about 40 months-losses in the average life expectancy. In 2050, the climate strategies reduce this indicator by 50 %. Decrease of ozone concentrations estimated for the climate scenario might save nearly 20,000 cases of premature death per year. Similarly significant are reductions of impacts on ecosystems due to acidification and eutrophication.  相似文献   
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127.
Soil microbes in temperate forest ecosystems are able to cycle several hundreds of kilograms of N per hectare per year and are therefore of paramount importance for N retention. Belowground C allocation by trees is an important driver of seasonal microbial dynamics and may thus directly affect N transformation processes over the course of the year. Our study aimed at unraveling plant controls on soil N cycling in a temperate beech forest at a high temporal resolution over a time period of two years, by investigating the effects of tree girdling on microbial N turnover. In both years of the experiment, we discovered (1) a summer N mineralization phase (between July and August) and (2) a winter N immobilization phase (November-February). The summer mineralization phase was characterized by a high N mineralization activity, low microbial N uptake, and a subsequent high N availability in the soil. During the autumn/winter N immobilization phase, gross N mineralization rates were low, and microbial N uptake exceeded microbial N mineralization, which led to high levels of N in the microbial biomass and low N availability in the soil. The observed immobilization phase during the winter may play a crucial role for ecosystem functioning, since it could protect dissolved N that is produced by autumn litter degradation from being lost from the ecosystem during the phase when plants are mostly inactive. The difference between microbial biomass N levels in winter and spring equals 38 kg N/ha and may thus account for almost one-third of the annual plant N demand. Tree girdling strongly affected annual N cycling: the winter N immobilization phase disappeared in girdled plots (microbial N uptake and microbial biomass N were significantly reduced, while the amount of available N in the soil solution was enhanced). This was correlated to a reduced fungal abundance in autumn in girdled plots. By releasing recently fixed photosynthates to the soil, plants may thus actively control the annual microbial N cycle. Tree belowground C allocation increases N accumulation in microorganisms during the winter which may ultimately feed back on plant N availability in the following growing season.  相似文献   
128.
Female birds might be able to manipulate the parental effort of their male partner through elevated transfer of hormones to the eggs, since these hormones affect many chick traits that males might use as cues for adjusting the level of their investment. We experimentally studied whether female pied flycatchers Ficedula hypoleuca could manipulate male investment via yolk androgens. There is much more variation in yolk androgen levels between females than within clutches, and in order to change the androgen levels of the eggs, we swapped whole clutches between nests. To estimate the androgen levels of the clutch, we measured the androgen content of a single egg per clutch. Females did not succeed in manipulating male effort using yolk androgens, since there was no relationship between the division of parental care within a pair and either original or foster egg androgen levels. One of these relationships should have occurred if females were manipulating males. The proportion of feeding visits by the male was higher when the male was old (55%) than when he was young (45%) and females laid eggs with higher androgen levels when mated with a young male. Young males did not exhibit any responses to yolk androgen levels either, which indicates that females cannot exploit their effort more than that of old males. We suggest that females may allocate yolk androgens to adjust the growth trajectories of the chicks to poor growing conditions when mated with young males that are poor providers or occupying a poor territory.  相似文献   
129.
Repetitive streamer discharges caused by transients, e.g. due to high frequency overvoltages, can ignite combustible mixtures, which has to be taken into account concerning the safety assessment of electrical apparatus for usage in hazardous areas. Hydrogen/air mixtures were ignited inside a closed vessel using a rod/plane electrode configuration. Alternating voltage with a frequency between 600 and 750 kHz and amplitudes of up to 20 kV was used to produce streamer discharges. The ignition process and the subsequent flame front propagation were examined with respect to mixture composition and several electrical parameters using time-resolved measurements of planar laser-induced fluorescence (PLIF) of OH radicals. A multiple pulse laser and detection system was used to assemble four images during one experiment. These measurements have given detailed information about the point of ignition and flame velocities. The experimental results will be used to validate numerical simulations of ignition by streamer discharges, which will yield deep insights into this specific ignition process.  相似文献   
130.
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