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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.
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   
5.
Miranda B  Díaz E  Ordóñez S  Vega A  Díez FV 《Chemosphere》2007,66(9):1706-1715
The performance of bulk chromium oxide is compared with that of a Mn commercial catalyst for the deep oxidation of trichloroethene (1000-2500ppmv, 55h(-1) space velocity) in air, in dry and wet (20000ppm of H(2)O) conditions, in terms of activity, selectivity and stability. Chromium oxide was found to be more active (on a catalyst weight basis), however its activity decreases continuously with time on stream. The presence of water increases its stability, the Mn catalyst showing the opposite behaviour. The effect of water on the Cr catalyst can be explained according to the Deacon equilibrium, as the presence of water tends to decrease the Cl(2) concentration, assumed to be responsible of the catalyst deactivation. Regarding to the selectivity, the Mn catalyst yields C(2)Cl(4), CCl(4) and CHCl(3) as organochlorinated by-products, whereas chromium oxide produces only trace amounts of CCl(4). Simple power-law kinetics expressions (first-order for Mn and zero-order for Cr) provide fairly good fits for the evolution of the conversion with the temperature. Furthermore, the kinetic behaviour of chromium oxide can be represented with a Langmuir-Hinshelwood model taking into account the chlorine inhibitory effect.  相似文献   
6.
The structural investigation and the chromium adsorptive potential of an exopolysaccharide (EPS) released during the growth of an indigenous cyanobacterium, Oscillatoria trichoides Szafer, were investigated in a laboratory‐scale study. The results showed that, of the total EPS produced, 410.53 milligrams/gram (mg g?1) were released polysaccharides (RPS) and 11.36 mg g?1 were capsular polysaccharides (CPS). The sorption of hexavalent chromium (Cr6+) by the RPS achieved a maximum amount of metal removal (qmax) value of 76.92 mg g?1 of polysaccharide dry weight. The highest coefficient of determination (0.9742) for the Langmuir adsorption model indicates best fitness of the model in explaining the sorption as a unilayer process. Equilibrium studies indicated that 30 to 40 milligrams per liter initial chromium concentration and a pH of 2 were optimal for biosorption of chromium by the RPS. Scanning electron microscopy with energy‐dispersive X‐ray spectroscopy analysis of Cr6+‐treated RPS showed the presence of 3.76% bound chromium. Compositional analysis of the EPS showed the presence of carbohydrates, proteins, pyruvic acid, and hexosamines. High‐performance liquid chromatography analysis demonstrated the presence of hexoses, as neutral sugars and glucuronic acid as an acidic sugar. The presence of carboxylic groups was also detected by infrared spectroscopy. The presence of these chemical constituents may serve as binding sites for the metal ions; therefore, the RPS of this species appears to be a promising biosorbent for Cr6+.  相似文献   
7.
The core element of the Costa Rican forestry policy is a financial instrument called the environmental service payment. This instrument rewards forest owners for the environmental services (the mitigation of greenhouse gases, the protection of watersheds and scenic beauty, and the development of biodiversity) their forests provide. In this article, the experiences with this new instrument are analyzed by focusing on the way interests are represented and access is granted, the openness of information exchange, whether social learning occurred, and whether decision-making authority is shared. The analysis is based on a survey conducted in the Huetar Norte Region and on in-depth interviews with the major stakeholders. The Costa Rican case indicates that financial instruments can be used to share responsibilities and that stakeholders can successfully cooperate on forest issues. It also shows that such a participatory approach is only promising if certain cultural, economic, organizational, and political conditions are met.  相似文献   
8.
The main aim of this study was to construct several regression models of air quality using techniques based on the statistical learning, in the metropolitan area of Oviedo, in northern Spain. In this research, a hybrid particle swarm optimization-based evolutionary support vector regression is implemented to predict the air quality from the experimental dataset (specifically, nitrogen oxides, carbon monoxide, sulfur dioxide, ozone, and dust) collected from 2013 to 2015 in the metropolitan area of Oviedo. Furthermore, a multilayer perceptron network (MLP) and the M5 model tree were also fitted to the experimental dataset for comparison purposes. Finally, the predicted results show that the hybrid proposed model is more robust than the MLP and M5 model tree prediction methods in terms of statistical estimators and testing performances.  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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