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2.
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   
3.
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   
4.
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   
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.
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   
7.
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   
8.
Semipermeable membrane devices (SPMDs) were deployed in streams along a gradient of urban land-use intensity in and around six metropolitan areas: Atlanta, Georgia; Raleigh-Durham, North Carolina; and Denver-Fort Collins, Colorado, in 2003; and Dallas-Fort Worth, Texas; Milwaukee-Green Bay, Wisconsin; and Portland, Oregon, in 2004 to examine relations between percent urban land cover in watersheds and the occurrence, concentrations, and potential toxicity of hydrophobic compounds. Of the 142 endpoints measured in SPMD dialysates, 30 were significantly (alpha = 0.05) related to the percent of urban land cover in the watersheds in at least one metropolitan area. These 30 endpoints included the aggregated measures of the total number of compounds detected and relative toxicity (Microtox(R) and P450RGS assays), in addition to the concentrations of 27 individual hydrophobic compounds. The number of compounds detected, P450RGS assay values, and the concentrations of pyrogenic polycyclic aromatic hydrocarbons (PAHs) were significantly related to percent urban land cover in all six metropolitan areas. Pentachloroanisole, the most frequently detected compound, was significantly related to urban land cover in all metropolitan areas except Dallas-Fort Worth. Petrogenic PAHs and dibenzofurans were positively related to percent urban land cover in Atlanta, Raleigh-Durham, Denver, and Milwaukee-Green Bay. Results for other endpoints were much more variable. The number of endpoints significantly related to urban land cover ranged from 6 in Portland to 21 Raleigh-Durham. Based on differences in the number and suite of endpoints related to urban intensity, these results provide evidence of differences in factors governing source strength, transport, and/or fate of hydrophobic compounds in the six metropolitan areas studied. The most consistent and significant results were that bioavailable, aryl hydrocarbon receptor agonists increase in streams as basins become urbanized. Potential toxicity mediated by this metabolic pathway is indicated as an important factor in the response of aquatic biota to urbanization.  相似文献   
9.
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   
10.
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   
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