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421.
Sanjad–Sakati syndrome (SSS) is an autosomal recessive disorder characterized by congenital hypoparathyroidism, growth and mental retardation. In Saudi Arabia, the disease is caused by a deletion of 12 bp (155-166nt) in the tubulin-specific chaperone E gene. In a family with two affected siblings with SSS, preimplantation genetic diagnosis (PGD) was performed. Fluorescent PCR (F-PCR) was utilized to check the heterozygosity and the homozygosity status of the parents and the affected children, respectively. F-PCR was then optimized for single-cell analysis by using peripheral blood lymphocytes. The patient underwent a cycle with intra-cytoplasmic sperm injection. A total of 11 embryos were obtained and biopsied. There were five heterozygous, three homozygous affected and three normal embryos. One heterozygous and one normal embryo were transferred because of their very good quality (morula). A singleton pregnancy was obtained, and amniosynthesis confirmed the presence of the heterozygous fetus. These results show for the first time, the feasibility of PGD for SSS. Copyright © 2004 John Wiley & Sons, Ltd. 相似文献
422.
《Journal of Manufacturing Processes》2014,16(2):166-175
Aluminum–lithium (Al–Li) alloys are very attractive for potential aerospace applications due to their lower density, higher specific strength and rigidity, better corrosion and fatigue crack growth resistance properties, compared to conventional aluminum alloys. Laser beam welding (LBW), being an advanced joining method, has already been approved for applications involving aluminum alloys. However, there are still a number of problems and issues to be answered and solved in LBW of Al–Li alloys. In this review, the properties of Al–Li alloys and the characteristics of LBW are introduced, the formation and prevention of the main weld defects such as porosity and hot cracking are discussed, and then the weld microstructure and the joint mechanical properties are described in highlight. At the end, an outlook on future trends is presented. 相似文献
423.
《Journal of Manufacturing Processes》2014,16(2):233-240
A thermo-mechanical model has been developed for modeling of hot extrusion processes. Accordingly, an admissible velocity field was first proposed by means of stream function method and then, extrusion pressure as well as temperature variations within the metal and the die were predicted employing a combined upper bound and Petrov–Galerkin finite element analysis. In order to evaluate the model predictions, hot extrusion of AA6061-10%SiCp was considered under both isothermal and non-isothermal conditions and the predicted force–displacement diagrams under various extrusion conditions were compared with the experimental ones and reasonable consistency was found between the two sets of results. 相似文献
424.
425.
Giuseppe Curcurù Giacomo Maria Galante Concetta Manuela La Fata 《Journal of Loss Prevention in the Process Industries》2013,26(6):1285-1292
The paper proposes an imprecise Fault Tree Analysis in order to characterize systems affected by the lack of reliability data. Differently from other research works, the paper introduces a classification of basic events into two categories, namely Initiators and Enablers. Actually, in real industrial systems some events refer to component failures or process parameter deviations from normal operating conditions (Initiators), whereas others refer to the functioning of safety barriers to be activated on demand (Enablers). As a consequence, the output parameter of interest is not the classical probability of occurrence of the top event, but its Rate of OCcurrence (ROCOF) over a stated period of time. In order to characterize the basic events, interval-valued information supplied by experts are properly aggregated and propagated to the top. To this purpose, the Dempster–Shafer Theory of evidence is proposed as a more appropriate mathematical framework than the classical probabilistic one. The proposed methodology, applied to a real industrial scenario, can be considered a helpful tool to support risk managers working in industrial plants. 相似文献
426.
J. Grune K. Sempert M. Kuznetsov T. Jordan 《Journal of Loss Prevention in the Process Industries》2013,26(6):1442-1451
This paper presents results of an experimental investigation on fast flame propagation and the deflagration-to-detonation transition (DDT) and following detonation propagation in a semi-confined flat layer filled with stratified hydrogen–air mixtures. The experiments were performed in a transparent, rectangular channel open from below. The combustion channel has a width of 0.3 m and a length of 2.5 m. The effective layer thickness in the channel was varied by using different linear hydrogen concentration gradients. The method to create quasi-linear hydrogen concentration gradients that differ in the range and slope is also presented. The ignited mixtures were accelerated quickly to sonic flame speed in the first obstructed part of the channel. The interaction of the fast flame propagation with different obstacle set-ups was studied in the second part of the channel. The experimental results show an initiation of DDT by one additional metal grid in the obstructed semi-confined flat layer. Detonation propagation and failed detonation propagation were observed in obstructed and unobstructed parts of the channel. 相似文献
427.
Kazimierz T. Kosmowski 《International journal of occupational safety and ergonomics》2013,19(3):276-293
This article addresses methodological issues of the human reliability analysis (HRA) in the context of probabilistic safety studies. Several conventional HRA techniques, more often used for the evaluation of the human error probabilities (HEPs), have been classified. A taxonomy of human actions, failure events, and related factors is outlined in order to distinguish action phases, human behavior types and incorrect outputs (errors of omission or commission), error types (slips, lapses, and mistakes), and performance-shaping factors (PSFs) influencing the human performance. A tree is proposed to facilitate the selection of a specific method for the evaluation of human reliability with regard to attributes of the situation analyzed. A software system based on the expert system technology to facilitate and document PSA and HRA is outlined. At the end of the article some research challenges in the domain are discussed. 相似文献
428.
Jonas Rønningsdalen Kunst Gøril Kvamme Løset Daniel Hosøy Bjørn Bjorvatn Bente E. Moen Nils Magerøy 《International journal of occupational safety and ergonomics》2013,19(1):139-147
The aim of the present study was to estimate spillover effects between the work and the family sphere in a sample of nurses (N = 2058). Hierarchical regression analyses investigated whether shift work schedules were associated with negative or positive spillover, both from family to work and vice versa, controlling for demographic factors, job demands and decision latitude. With daytime work as a reference group, all types of shift work (day and evening shift, night shift only and rotating 3 shift) were associated with higher negative work-to-family spillover. Night work was associated with significantly less negative family-to-work spillover. None of the different shift work schedules were related to any type of positive spillover. The results indicate that working outside of daytime hours is less compatible with workers’ family lives, compared to working ordinary day shifts. On the other hand, working night shifts only was associated with reduced negative family-to-work spillover. 相似文献
429.
《Environmental Communication: A Journal of Nature and Culture》2013,7(4):520-538
Aron Ralston and Christopher McCandless, two outdoor adventurers, have captured the hearts of many American environmentalists. Each has attained the status of cultural icons and inspired books and films to recount their tales. While entertainment media have romanticized both individuals, news media are not as easy on one—McCandless is vilified while Ralston is valorized. When reporting these stories, news media outlets attempted to retell each story in a way that conforms to the dominant American ideology of wilderness, where “progress” is marked by control over nature—control that both Ralston and McCandless clearly unsettled. In addition, both committed many of the same errors in being underprepared. Despite these similarities, why are they each presented so differently in the news media? This paper offers a rhetorical analysis of newspaper articles on each story, where the phenomenon of scapegoating alienates McCandless, and the phenomenon of mortification purifies Ralston, restabilizing this American environmental ideology in both stories. In conclusion, I argue that the essential difference between these two stories is that they present two opposing ideals of a human–nature relationship, with Ralston's ideology including a space for technology and industrial knowledge, and thus construed as more appropriate than McCandless' ideology. 相似文献
430.
《International Journal of Sustainable Engineering》2013,6(2):135-144
The contribution contained in this paper focuses on providing a possible framework or approach in respect of how manufacturing companies can economically introduce environmentally friendly practices to their production operations, whilst at the same time encouraging organisational and operational learning with the aim of eventual evolution of the firm into an eco-efficient concern. A key premise associated with the approach advocated is economical organisational and operational learning over time, thus providing a sequential movement of the company through various stages of cultural change and technological capability to eventually achieve eco-efficiency in its production activities. At each stage in the process, it is suggested that a balance must be maintained between on the one hand, a reduction in operational economics consistent with on the other hand, an improvement in the environmental sustainability of company activities. To that end, some previously suggested monitoring metrics are examined in the text for their efficacy and economic rapport via hypothetical examples of how they might be applied in practice to monitor movement towards industrial–environmental sustainability. 相似文献