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81.
Two patients with biotinidase deficiency had diagnoses of infantile spasms made at 1 month of age. Biotinidase deficiency may be seen early in the neonatal period without the characteristic findings such as alopecia and seborrheic dermatitis. This diagnosis should be considered in patients with infantile spasms.  相似文献   
82.
For successful data delivery, the destination nodes should be listening to the medium to receive data when the sender node starts data communication. To achieve this synchronization, there are different rendezvous schemes, among which the most energy-efficient is utilizing wakeup receivers. Current hardware technologies of wake-up receivers enable us to evaluate them as a promising solution for wireless sensor networks. In this article the benefits achieved with wake-up receivers are investigated along with the challenges observed. In addition, an overview of state-of-the-art hardware and networking protocol proposals is presented. As wake-up receivers offer new opportunities, new potential application areas are also presented and discussed.  相似文献   
83.
Citric acid (CA) productivity by Yarrowia lipolytica dependents on strain type, carbon source, carbon to nitrogen (C/N) molar ratio as well as physicochemical conditions (pH, temperature, oxygen transfer rate, etc.). In the current study, 10 different Ylipolytica strains were first challenged in shake-flask culture for CA production in a glucose-based media under nitrogen-limited conditions. For the most promising one, strain K57, CA productivity was monitored during culture in batch bioreactor for three initial C/N molar ratio (167, 367, and 567 Cmol/Nmol). The highest CA yield (0.77 g/g glucose), titre (72.3 g/L CA), and productivity (0.04 g/g.h) were found for C/N ratio of 367. However, the highest growth rate was obtained for an initial C/N ratio of 167. From these results, Ylipolytica strain K57 could be considered to produce CA at higher titre on glucose-based medium in batch bioreactor than others Ylipolytica strain reported in the literature.  相似文献   
84.
This paper aims to determine the factors that have an impact on the consumers’ willingness to pay a premium for hybrid automobiles in Turkey. A web-based random survey was conducted in different regions of Turkey. A questionnaire was administered to 1983 participants in January–March of 2009. The questionnaire was prepared by taking the issues raised in various sources into account. An ordered Probit model was used to meet the objective. Results show that variables such as income, gender, education, concerns about global warming, number of automobiles, importance of automobile performance, risk preference, attitude toward the alternative energy sources have an impact on the consumers’ willingness to pay a premium for hybrids. Findings suggest that consumers who have high income, higher educational level, and concerns about the global warming are more likely to pay a premium for hybrids. This study is expected to make important contributions to the current literature related to the consumers’ willingness to pay for hybrids by providing a research study from a developing country’s perspective. Results of this study also make important contributions to the policy and decision makers, environmental groups and automotive industry.  相似文献   
85.
A new computational method based on the J k -integral is put forward for the purpose of conducting fracture analysis of orthotropic materials subjected to hygrothermal stresses. By utilizing the constitutive relations of plane orthotropic hygrothermoelasticity, an alternative expression for the J k -integral is derived to replace the general limit definition. A numerical procedure is developed and integrated into a finite element analysis software to implement the proposed form of the J k -integral. Temperature and specific moisture concentration fields, which are required in fracture calculations, are also computed through finite element analysis. Numerical results are generated by considering an embedded crack in a polymer matrix fibrous composite laminate, that is subjected to steady-state hygrothermal loading. Comparisons of the mixed-mode stress intensity factors computed by the J k -integral based method to those evaluated via the displacement correlation technique demonstrate that, the proposed form of the J k -integral is domain independent and leads to numerical results of high accuracy. Presented parametric analyses illustrate the influences of the fiber volume fraction and the crack location on the modes I and II stress intensity factors, the energy release rate, and the T-stress.  相似文献   
86.
Buildings play a key role in total world energy consumption as a consequence of poor thermal insulation characteristics of facade materials. Among the elements of a typical building envelope, windows are responsible for the greatest energy loss because of their notably high overall heat transfer coefficients. About 60% of heat loss through the building fabric can be attributed to the glazed areas. In this respect, novel cost‐effective glazing technologies are needed to mitigate energy consumption, and thus to achieve the latest targets toward low/zero carbon buildings. Therefore in this study, three unique glazing products called vacuum tube window, heat insulation solar glass and solar pond window which have recently been developed at the University of Nottingham are introduced, and thermal performance analysis of each glazing technology is done through a comparative experimental investigation for the first time in literature. Standardized co‐heating test methodology is performed, and overall heat transfer coefficient (U‐value) is determined for each glazing product following the tests carried out in a calibrated environmental chamber. The research essentially aims at developing cost‐effective solutions to mitigate energy consumption because of windows. The results indicate that each glazing technology provides very promising U‐values which are incomparable with conventional commercial glazing products. Among the samples tested, the lowest U‐value is obtained from the vacuum tube window by 0.40 W/m2K, which corresponds to five times better thermal insulation ability compared to standard air filled double glazed windows. Copyright © 2015 John Wiley & Sons, Ltd.  相似文献   
87.
The water gas shift activity of promoted Pt–CeO2/Al2O3 catalysts were investigated in this work. The catalysts were prepared by incipient to wetness impregnation and tested using a microflow reaction system. It was found that K has beneficial effects under product-containing feed compositions while Co and Ni promoters worsen catalyst performance. The reaction temperature and feed H2O/CO ratio positively affect the catalytic activity, whereas CO2 and H2 addition to the feed decreases CO conversion, as expected. The experimental results were also modeled using modular neural networks, at which the catalyst preparation and operational (reaction) variables were used together in the same network because they are interacting but processed differently because they are dissimilar in their form (i.e. categorical versus continuous) and their effects on catalytic activity. It was concluded that the effects of catalyst preparation and operational variables and their relative importance could be comprehended more accurately by using this approach, which may be also employed in other similar systems.  相似文献   
88.
In this study, an air window collector having vertical black blinds was investigated experimentally under actual outdoor conditions. The vertical blind was installed between two 120 × 80 cm glass panes. It can rotate about the vertical axis. The fully open position makes a 90° angle with the vertical plane. A commercially available blind made of cloth and coated with black paint was tested. The air circulation in the system is provided by a fan parallel to blinds. Various parameters were measured and recorded using a data acquisition system. The effect of these parameters on the thermal performance of the system were determined experimentally.  相似文献   
89.
In this study, experimental and three-dimensional numerical studies were performed to investigate the effects of plate spacing and temperature difference on natural convection between isothermally heated upward-facing lower horizontal plate and externally insulated horizontal upper plate. Air is used as the heat transfer medium. Rayleigh number varied in the 1108–2.339 × 105 range. Several numerical simulations for three-dimensional steady laminar and turbulent flows heat transfer were carried out using a commercial CFD code Fluent 6.2. Results have shown that there is a good agreement between the numerical and present experimental results as well as with available data in literature.  相似文献   
90.
Due to its high hydrogen density and extensive experience base, ammonia (NH3) has been gaining special attention as a potential green energy carrier. This study focuses on premixed ammonia–hydrogen–air flames under standard temperature and pressure conditions using an inert silicon-carbide (SiC) porous block as a practical and effective medium for flame stabilization. Combustion experiments conducted using a lab scale burner resulted in stable combustion and high combustion efficiencies at very high ammonia concentration levels over a wide range of equivalence ratios. Noticeable power output densities have also been achieved. Preliminary results of NOx emission measurements indicate NOx concentrations as low as 35 ppm under rich conditions. The remarkable capability of this specific burner to operate efficiently and cleanly at high ammonia concentration levels, which can easily be achieved by partial cracking of NH3, is believed to be a key accomplishment in the development of ammonia fired power generation systems.  相似文献   
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