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61.
Chondroectodermal dysplasia (Ellis-van Creveld syndrome) has previously been diagnosed prenatally only once, using fetoscopy. We report on two consecutive pregnancies in a woman at risk of having a child with the syndrome during which fetoscopic visualization was performed. Ellis-van Creveld syndrome was diagnosed prenatally in one instance, while it could be excluded in the other one. Non-invasive prenatal diagnosis of the syndrome is discussed. 相似文献
62.
At 10 weeks' gestation, chorionic villus biopsy was obtained from a woman at risk for Sanfilippo type C disease. Acetyl-CoA: α-glucosaminide N-acetyltransferase activity was markedly deficient. The pregnancy was terminated at 12 weeks' gestation and follow-up study on fetal fibroblasts confirmed the diagnosis. 相似文献
63.
Azo compounds are widely involved in the industrial processes of dyes, pigments, initiators, and blowing agents. Unfortunately, these compounds have a bivalent unstable –NN– composition, which can be readily broken when the ambient temperature is elevated. Self-accelerating decomposition might cause a runaway reaction and lead to a fire, explosion, or leakage when the cooling system fails or other events occur. This study investigated the explosion properties, thermal stability parameters, and thermal hazard and mechanism of 2,2′–azobisisobutyronitrile (AIBN) and 2,2′–azobis–2–methylbutyronitrile (AMBN). We used a 20-L apparatus, vent sizing package 2, synchronous thermal analysis, and differential scanning calorimetry under explosive, adiabatic, and dynamic conditions to acquire the explosive curves, thermal curves, and thermodynamic parameters of the substances. Moreover, the differential isoconversional method (Friedman method) and ASTM E698 equation were employed to obtain the apparent activation energy Ea. All the experimental results revealed that AIBN is more dangerous than AMBN. The Ea of AIBN was lower than that of AMBN. The results can be used to construct an azo compound thermal hazard database for use for searches and reference examples by industry and related research areas. 相似文献
64.
A. Ben Hassen-Trabelsi T. Kraiem S. Naoui H. Belayouni 《Waste management (New York, N.Y.)》2014,34(1):210-218
Several animal (lamb, poultry and swine) fatty wastes were pyrolyzed under nitrogen, in a laboratory scale fixed-bed reactor and the main products (liquid bio-oil, solid bio-char and syngas) were obtained. The purpose of this study is to produce and characterize bio-oil and bio-char obtained from pyrolysis of animal fatty wastes. The maximum production of bio-oil was achieved at a pyrolysis temperature of 500 °C and a heating rate of 5 °C/min. The chemical (GC–MS analyses) and spectroscopic analyses (FTIR analyses) of bio-oil showed that it is a complex mixture consisting of different classes of organic compounds, i.e., hydrocarbons (alkanes, alkenes, cyclic compounds…etc.), carboxylic acids, aldehydes, ketones, esters,…etc. According to fuel properties, produced bio-oils showed good properties, suitable for its use as an engine fuel or as a potential source for synthetic fuels and chemical feedstock. Obtained bio-chars had low carbon content and high ash content which make them unattractive for as renewable source energy. 相似文献
65.
Pierre Hennebert Hans A. van der Sloot Flore Rebischung Reinhilde Weltens Lieve Geerts Ole Hjelmar 《Waste management (New York, N.Y.)》2014,34(10):1739-1751
Hazard classification of waste is a necessity, but the hazard properties (named “H” and soon “HP”) are still not all defined in a practical and operational manner at EU level. Following discussion of subsequent draft proposals from the Commission there is still no final decision. Methods to implement the proposals have recently been proposed: tests methods for physical risks, test batteries for aquatic and terrestrial ecotoxicity, an analytical package for exhaustive determination of organic substances and mineral elements, surrogate methods for the speciation of mineral elements in mineral substances in waste, and calculation methods for human toxicity and ecotoxicity with M factors.In this paper the different proposed methods have been applied to a large assortment of solid and liquid wastes (>1 0 0).Data for 45 wastes – documented with extensive chemical analysis and flammability test – were assessed in terms of the different HP criteria and results were compared to LoW for lack of an independent classification. For most waste streams the classification matches with the designation provided in the LoW. This indicates that the criteria used by LoW are similar to the HP limit values.This data set showed HP 14 ‘Ecotoxic chronic’ is the most discriminating HP. All wastes classified as acute ecotoxic are also chronic ecotoxic and the assessment of acute ecotoxicity separately is therefore not needed. The high number of HP 14 classified wastes is due to the very low limit values when stringent M factors are applied to total concentrations (worst case method). With M factor set to 1 the classification method is not sufficiently discriminating between hazardous and non-hazardous materials. The second most frequent hazard is HP 7 ‘Carcinogenic’. The third most frequent hazard is HP 10 ‘Toxic for reproduction’ and the fourth most frequent hazard is HP 4 “Irritant – skin irritation and eye damage”. In a stepwise approach, it seems relevant to assess HP 14 first, then, if the waste is not classified as hazardous, to assess subsequently HP 7, HP 10 and HP 4, and then if still not classified as hazardous, to assess the remaining properties.The elements triggering the HP 14 classification in order of importance are Zn, Cu, Pb, Cr, Cd and Hg. Progress in the speciation of Zn and Cu is essential for HP 14. Organics were quantified by the proposed method (AFNOR XP X30-489) and need no speciation. Organics can contribute significantly to intrinsic toxicity in many waste materials, but they are only of minor importance for the assessment of HP 14 as the metal concentrations are the main HP 14 classifiers. Organic compounds are however responsible for other toxicological characteristics (hormone disturbance, genotoxicity, reprotoxicity…) and shall be taken into account when the waste is not HP 14 classified. 相似文献
66.
Brian E. Reichert Mylea Bayless Tina L. Cheng Jeremy T. H. Coleman Charles M. Francis Winifred F. Frick Benjamin S. Gotthold Kathryn M. Irvine Cori Lausen Han Li Susan C. Loeb Jonathan D. Reichard Thomas J. Rodhouse Jordi L. Segers Jeremy L. Siemers Wayne E. Thogmartin Theodore J. Weller 《Ambio》2021,50(4):901
Collaborative monitoring over broad scales and levels of ecological organization can inform conservation efforts necessary to address the contemporary biodiversity crisis. An important challenge to collaborative monitoring is motivating local engagement with enough buy-in from stakeholders while providing adequate top-down direction for scientific rigor, quality control, and coordination. Collaborative monitoring must reconcile this inherent tension between top-down control and bottom-up engagement. Highly mobile and cryptic taxa, such as bats, present a particularly acute challenge. Given their scale of movement, complex life histories, and rapidly expanding threats, understanding population trends of bats requires coordinated broad-scale collaborative monitoring. The North American Bat Monitoring Program (NABat) reconciles top-down, bottom-up tension with a hierarchical master sample survey design, integrated data analysis, dynamic data curation, regional monitoring hubs, and knowledge delivery through web-based infrastructure. NABat supports collaborative monitoring across spatial and organizational scales and the full annual lifecycle of bats. 相似文献
67.
This research seeks for improved understanding regarding the interaction of meaningful work and the work–family interface. Existing literature suggests that experiencing a sense of calling toward work makes the work domain particularly salient to employees compared to other life domains. In this article, we draw on this idea, rooted in identity theory, to hypothesize that a sense of calling toward work diminishes the effects of work–family conflict and work–family enrichment on employee's job and life satisfaction. We test these ideas in two studies. First, we surveyed an alumni sample of 598 employees from various jobs, industries, and job levels. Then, in a constructive replication, we surveyed 327 employees using a time-lagged design. Calling was found to significantly buffer the effect of work–family conflict on job satisfaction in Study 2, but not Study 1. Calling did not buffer the effect of conflict on life satisfaction in either study. However, both studies demonstrated that calling attenuated (substituted for) the effect of work–family enrichment on job satisfaction. Study 1 supported the idea that calling attenuates the effect of enrichment on life satisfaction; however, this interactive effect was reversed in Study 2, contrary to expectations. We discuss implications for theory and practice related to callings and career choices, as well as for the role of calling and work identity in the work–family interface. 相似文献
68.
Lea Nicita Giovanni Signorello Maria De Salvo 《Journal of Environmental Planning and Management》2016,59(7):1225-1237
This paper applies the Kuhn–Tucker model to estimate recreation demand of parks in Sicily. We estimate a fixed coefficient specification and a random coefficient specification to take into account heterogeneity across visitors. Estimates suggest a diversity of preferences across the population and that parks with higher level of quality attributes are more likely to be visited. We also simulate two sets of hypothetical policy scenarios to evaluate and compare the recreational value of each park and the welfare impacts of changes in a quality attribute. 相似文献
69.
A conceptual approach to model sand–gravel extraction from rivers based on a game theory perspective
Marianthi V. Podimata 《Journal of Environmental Planning and Management》2016,59(1):120-141
Sand–gravel mining is a significant parameter of economic development and social welfare function in modern societies. As demand for aggregate increases in construction industry, conflicts for the availability of the resource and environmental impacts become more intense. The present paper describes the contested status quo in riverbed sand–gravel mining activities with an example from Greece, as a case study. The scope is to propose a methodology about good governance of the mining sector that promotes a sustainable sharing of aggregate resource by securing environment and safekeeping revenues in the mining trade market. 相似文献
70.
A combined photovoltaic–thermal (PV/t) panel is proposed to produce simultaneously electricity and heat from one integrated unit. The unit utilizes effectively the solar energy through achieving higher PV electrical efficiency and using the thermal energy for heating applications. To predict the performance of the PV/t at a given environmental conditions, a transient mathematical model was developed. The model was integrated in a heating application for a typical office space in the city of Beirut to provide the office needs for electricity, heating during winter season, and dehumidification and evaporative cooling during the summer season. To minimize the yearly office energy (electrical and heat) needs, the PV/t panel cooling air flow rate and the dehumidification regeneration temperature were determined for opimal unit operation. Thermal energy savings of up to 85% in winter and 71% in summer were achived compared to conventional systems at a payback period of 8 years for the panels. 相似文献