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Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is a potentially fatal inherited disease with a carrier frequency of approximately 1:100 in most Caucasian populations. The disease is implicated in sudden unexpected death in childhood. A prevalent disease-causing point mutation (A985G) in the MCAD gene has been characterized, thus rendering diagnosis easy in the majority of cases. Since the clinical spectrum of MCAD deficiency ranges from death in the first days of life to an asymptomatic life, there are probably other genetic factors—in addition to MCAD mutations—involved in the expression of the disease. Thus, families who have experienced the death of a child from MCAD deficiency might have an increased risk of a seriously affected subsequent child. In such a family we have therefore performed a prenatal diagnosis on a chorionic villus sample by a highly specific and sensitive polymerase chain reaction (PCR) assay for the G985 mutation. The analysis was positive and resulted in abortion. We verified the diagnosis by direct analysis on blood spots and other tissue material from the aborted fetus and from family members.  相似文献   
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Hydrogen storage and transportation or distribution is closely linked together. Hydrogen can be distributed continuously in pipelines or batch wise by ships, trucks, railway or airplanes. All batch transportation requires a storage system but also pipelines can be used as pressure storage system. Hydrogen exhibits the highest heating value per weight of all chemical fuels. Furthermore, hydrogen is regenerative and environment friendly. There are two reasons why hydrogen is not the major fuel of toady’s energy consumption: First of all, hydrogen is just an energy carrier. And, although it is the most abundant element in the universe, it has to be produced, since on earth it only occurs in the form of water. This implies that we have to pay for this energy, which results in a difficult economic task, because since the industrialization we are used to consuming energy for free. The second difficulty with hydrogen as an energy carrier is the low critical temperature of 33 K, i.e. hydrogen is a gas at room temperature. For mobile and in many cases also for stationary applications the volumetric and gravimetric density of hydrogen in a storage system is crucial. Hydrogen can be stored by six different methods and phenomena: high pressure gas cylinders (up to 800 bar), liquid hydrogen in cryogenic tanks (at 21 K), adsorbed hydrogen on materials with a large specific surface area (at T < 100 K), absorbed on interstitial sites in a host metal (at ambient pressure and temperature), chemically bond in covalent and ionic compounds (at ambient pressure), oxidation of reactive metals e.g. Li, Na, Mg, Al, Zn with water. These metals easily react with water to the corresponding hydroxide and liberate the hydrogen from the water. Finally, the metal hydroxides can be thermally reduced to the metals in a solar furnace.  相似文献   
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The reestablisment of autochthonous plant species is an essential strategy for recovering degraded areas under semiarid conditions. A field experiment was carried out to assess the short-term effect of two reafforestation methods involving mycorrhizal inoculation and compost addition on soil quality parameters and Rhamnus lycioides seedling growth. The nutrient content (NPK) and enzymatic activities (dehydrogenase, urease, protease-BAA, acid phosphatase and β-glucosidase) increased and bulk density decreased in the rhizosphere soil with the organic amendment. Biomass C of rhizosphere soil increased by at least 240% with respect to the control soil after mycorrhizal inoculation and the combination of compost addition + mycorrhizal inoculation. Both mycorrhizal inoculation and composted organic residue addition increased R. lycioides seedling growth in the same proportion. In the short term, we conclude that the application of both reafforestation methods not only enhances the establishment of R. lycioides seedlings, but also improves soil quality.  相似文献   
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Methane oxidation in slurry storage surface crusts   总被引:1,自引:0,他引:1  
Livestock manure is a significant source of atmospheric methane (CH4), especially during liquid storage. In liquid manure (slurry) storages a surface crust may form naturally, or an artificial surface crust can be established. We investigated whether there is a potential for CH4 oxidation in this environment. Surface crust materials were sampled from experimental storages with cattle slurry (with natural crust) or anaerobically digested cattle slurry (with straw layer) that had been stored with or without a wooden cover. Extracts of surface crust material were incubated with 5.6% CH4 in the headspace, and methanotrophic activity was demonstrated in all four treatments following a 4- to 10-d lag phase. Subsequent incubation of field-moist surface crust material with 350 microL L(-1) CH4 also showed CH4 oxidation, indicating a potential for CH4 removal under practical storage conditions. There was no CH4 oxidation activity during incubation of autoclaved samples. Methane oxidation rates were 0.1 to 0.5 mg kg(-1) organic matter (OM) h(-1), which is comparable with the activity in wetlands and rice paddies. Partial drying increased CH4 oxidation to 0.2 to 1.4 mg kg(-1) OM h(-1), probably as a result of improved diffusivity within the surface crust. Rewetting reversed the stimulation of methanotrophic activity in some treatments, but not in others, possibly due to a decline in CH4 production in anaerobic volumes, or to growth of methanotrophs during incubation. This study presents direct evidence for methanotrophic activity in slurry storages. Measures to ensure crust formation with or without a solid cover appear to be a cost-effective greenhouse gas mitigation option.  相似文献   
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This study aimed at finding effective strategies for high-performance removal of reactive blue 19 (RB19) dye from aqueous solution. Chitosan (CS) films had been prepared by using solvent casting with mild drying for this purpose. The CS films were characterized by X-ray diffraction, field-emission scanning electron microscopy, and Fourier transform infrared (FTIR) spectroscopy. The performance of RB19 removal using CS were evaluated by varying contact time, solution pH, initial dye concentration, and adsorbent dosage. Adsorption isotherms, kinetics, and desorption were investigated by batch experiments. Results showed that CS films exhibited the optimal adsorption performance for RB19 removal and high maximum adsorption capacities of RB19, which were 799 and 822.4 mg g?1 at 20 and 40 °C, respectively. Adsorption kinetic data were well described by the pseudo-second-order kinetic model. FTIR analyses further indicated that interactions between RB19 and the CS film occurred during adsorption. The CS films also exhibited satisfactory desorption of RB19 at about 80 % after 30 min of desorption at pH 11. Our study demonstrated that the CS films can be easily prepared and applied for effective removal of RB19 in treatment of wastewater.  相似文献   
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