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1.
A case of fatal generalized dystrophic epidermolysis bullosa is described in a prematurely born female whose mother had strikingly elevated mid-trimester serum and amniotic fluid alpha-fetoprotein concentrations, a positive amniotic fluid acetylcholinesterase band, and negative serial ultrasound studies. This case lends further support to an association between autosomal recessive generalized dystrophic epidermolysis bullosa and increased levels of alpha-fetoprotein, positive amniotic fluid acetylcholines'terase, and normal ultrasound findings.  相似文献   
2.
A dicentric X chromosome was found in a female fetus during cytogenetic studies performed on amniotic cells. Blood samples from the parents showed normal karyotypes and the pregnancy was terminated. The mechanism for the formation of this ‘de novo’ rearrangement is discussed.  相似文献   
3.
Prenatal diagnosis in a pregnancy at risk for a juvenile B1 variant of GM2-gangliosidosis was carried out. The biochemical study of the cultured amniocytes and the affected fetal brain is reported. The results obtained show that the sulphated artificial substrate can be used in the diagnosis of B1 variant, but not the neutral one. The accumulation of GM2-ganglioside in the fetal brain of the B1 juvenile form and an infantile form of GM2-gangliosidosis (0 variant) was compared.  相似文献   
4.
Electrofishing is widely used to monitor fish species composition and relative abundance in streams and lakes. According to standard protocols, multiple segments are selected in a body of water to monitor population relative abundance as the ratio of total catch to total sampling effort. The standard protocol provides an assessment of fish distribution at a macrohabitat scale among segments, but not within segments. An ancillary protocol was developed for assessing fish distribution at a finer scale within electrofishing segments. The ancillary protocol was used to estimate spacing, dispersion, and association of two species along shore segments in two local reservoirs. The added information provided by the ancillary protocol may be useful for assessing fish distribution relative to fish of the same species, to fish of different species, and to environmental or habitat characteristics.  相似文献   
5.
Mercury (Hg) is a toxic element that is emitted to the atmosphere by both human activities and natural processes. Volcanic emissions are considered a natural source of mercury in the environment. In some cases, tree ring records taken close to volcanoes and their relation to volcanic activity over time are contradictory. In 1949, the Hoyo Negro volcano (La Palma-Canary Islands) produced significant pyroclastic flows that damaged the nearby stand of Pinus canariensis. Recently, 60 years after the eruption, we assessed mercury concentrations in the stem of a pine which survived volcano formation, located at a distance of 50 m from the crater. We show that Hg content in a wound caused by pyroclastic impacts (22.3 μg kg?1) is an order of magnitude higher than the Hg concentrations measured in the xylem before and after the eruption (2.3 μg kg?1). Thus, mercury emissions originating from the eruption remained only as a mark—in pyroclastic wounds—and can be considered a sporadic and very high mercury input that did not affect the overall Hg input in the xylem. In addition, mercury contents recorded in the phloem (9.5 μg kg?1) and bark (6.0 μg kg?1) suggest that mercury shifts towards non-living tissues of the pine, an aspect that can be related to detoxification in volcanism-adapted species.  相似文献   
6.
This article reflects on the Costa Rican experiences with ecotourism by assessing the positive and negative environmental, economic and social impacts of ecotourism development at four tourist destinations—Manuel Antonio, Monteverde, Tortuguero and ASCOMAFOR. These destinations represent different stages of tourism development. The assessment shows that the development of ecotourism has a dilemma character. Compared to alternative land-use options, ecotourism remains a promising development strategy. However, it should be embedded in a broader process of capacity building.  相似文献   
7.
In this study of 43 student project teams, we tested a multi‐level mediation model of the relationship between dissimilarity in conscientiousness, team temporal processes, and team member satisfaction. We distinguished between individual‐level dissimilarity in conscientiousness (i.e., the distance between an individual member and his or her team mates), and team‐level dissimilarity in conscientiousness (i.e., the overall dissimilarity within the team). Individual‐level dissimilarity in conscientiousness had a direct negative effect on team members' satisfaction with the team, but did not affect their satisfaction with the team's performance. Team‐level dissimilarity in conscientiousness indirectly affected both types of satisfaction negatively as it impeded early agreement about the temporal aspects of task execution, which, in turn, hindered coordinated action in later stages of team task execution. Copyright © 2008 John Wiley & Sons, Ltd.  相似文献   
8.
The disposal problem associated with phytoextraction of farmland polluted with heavy metals by means of willow requires a biomass conversion technique which meets both ecological and economical needs. Combustion and gasification of willow require special and costly flue gas treatment to avoid re-emission of the metals in the atmosphere, whereas flash pyrolysis mainly results in the production of (almost) metal free bio-oil with a relatively high water content. Flash co-pyrolysis of biomass and waste of biopolymers synergistically improves the characteristics of the pyrolysis process: e.g. reduction of the water content of the bio-oil, more bio-oil and less char production and an increase of the HHV of the oil. This research paper investigates the economic consequences of the synergistic effects of flash co-pyrolysis of 1:1 w/w ratio blends of willow and different biopolymer waste streams via cost-benefit analysis and Monte Carlo simulations taking into account uncertainties. In all cases economic opportunities of flash co-pyrolysis of biomass with biopolymer waste are improved compared to flash pyrolysis of pure willow. Of all the biopolymers under investigation, polyhydroxybutyrate (PHB) is the most promising, followed by Eastar, Biopearls, potato starch, polylactic acid (PLA), corn starch and Solanyl in order of decreasing profits. Taking into account uncertainties, flash co-pyrolysis is expected to be cheaper than composting biopolymer waste streams, except for corn starch. If uncertainty increases, composting also becomes more interesting than flash co-pyrolysis for waste of Solanyl. If the investment expenditure is 15% higher in practice than estimated, the preference for flash co-pyrolysis compared to composting biopolymer waste becomes less clear. Only when the system of green current certificates is dismissed, composting clearly is a much cheaper processing technique for disposing of biopolymer waste.  相似文献   
9.
Wood is commonly used in residential combustion for heating purposes; however, it can be a major source of air pollutants, namely fine particles, volatile organic compounds and carbon monoxide. Since 2004, the PM10 daily limit value has been surpassed in Portugal, and the European Commission has stated that plans and programs must be designed in order to reduce these levels. In Portugal, 18% of PM10 emissions are due to residential wood combustion, which may deeply impact the PM10 levels in the atmosphere. The main aim of this study is to investigate the impact of residential wood combustion on the air quality in Portugal. The air quality modelling system MM5/CHIMERE was applied over Portugal for a winter month, for the following three scenarios: the reference scenario, considering the actual emissions of PM10; scenario 1, where residential wood combustion emissions are not considered; and scenario 2, which takes into account a complete conversion from traditional fireplaces to certified appliances (with a 90% reduction in PM emissions). The residential wood combustion contribution to PM10 air quality concentration values during January 2007 ranges from 0 to 14 μg m?3, with a mean contribution of 10 μg m?3 in the Lisboa area and 6 μg m?3 in the Porto region. Concerning the legislated values, the area where the daily average limit value (50 μg m?3) is exceeded decreases by 46% in the simulation when residential combustion is not considered. The modelling results for scenario 2 are not significantly different from those for scenario 1. In summary, the regulation of the residential wood combustion sector is as an effective way to reduce the PM10 levels in the atmosphere as regards air quality plans and programs.  相似文献   
10.
The recovery and utilization of recovered paper have increased over past decades all over the world due to economic, environmental, and social issues. However, it is well known that an extended recovered paper collection is detrimental to its quality, either by the exploitation of lower quality sources such as households, or the spreading of commingled systems instead of selective collection systems. The influence of these two factors was assessed by analyzing the quality of different recovered paper grades used as raw material in a mill located in Madrid (Spain) producing newsprint and light weight coated paper from recovered paper. Part 1 of the paper deals with the impact of increased collection rates on the quality of recovered paper and Part 2 with the use of commingled collection systems. Results of Part 1 show that increased collection rates have a large impact on the quality of the recovered paper. The quality, measured as total unusable material and moisture contents, had deteriorated very rapidly in only 4 years (2005–2008) as a consequence of increased collection rates. Collection rates increased in Spain from 58.5% to 68.6% during this period, resulting in more than 50% increase of total unusable material and 25% of moisture content. The downgrading of the quality of recovered paper is one of the major threats for extending the current limits of paper recycling. Therefore, future challenge is to increase its availability but maintaining its quality.  相似文献   
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