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181.
Intrauterine dilation of critical fetal aortic stenosis (AS) and pulmonary stenosis or atresia has the potential to change the natural course of these congenital heart defects preventing progression to a single ventricle circulation. This article reviews the world experience in fetal cardiac interventions. In carefully selected cases, fetal cardiac surgery can reverse end-stage heart failure and can provide biventricular outcome postnatally in about two thirds of the cases with successful interventions. Copyright © 2011 John Wiley & Sons, Ltd.  相似文献   
182.
SMPD4 loss is a rare neurodevelopmental disorder that leads to severe mental and physical disability and early death in infancy. Most cases of this genetic condition have been diagnosed postnatally. This article focuses on the prenatal findings of affected fetuses. The phenotypes can include growth restriction, arthrogryposis (clenched hands, foot deformity), as well as cerebral abnormalities (simplified gyral pattern/lissencephaly, cerebellar hypoplasia, corpus callosum deformity). SMPD4 loss is detectable via exome sequencing. Here, two fetuses displayed a homozygotic pathogen variant in the SMPD4 gene, encoding for the enzyme Sphingomyelinase-4. Both parents were heterozygous carriers of the pathogenic variant. On detection of the above mentioned signs exome sequencing is indicated, with focus on the SMPD4 gene.  相似文献   
183.
我们于1988~2002年对未孵化的苍鹰卵(n=57)进行了调查,并与1971~1978年的卵(n=134)进行了比较,早期PCBs、∑DDT和HCB的浓度为(mg/kg脂重)50.1、126.6和117.7,而后期相应的值显著低,分别为27.7、4.1和0.1.但最近8~10年内,PCB的浓度相对稳定.残留物浓度波动在巢内低于巢间.年龄不影响卵中污染物的积累.1971~1978年测得的卵壳指数显著低,而2001~2002年测得的指数却相当高,与18世纪末的数字相当.我们提出了卵壳指数效应浓度的临界值.DDT族残留物对每处鸟巢的离巢幼鸟数目有影响.  相似文献   
184.
This paper provides an analysis of co-benefits for traditional air pollutants made possible through global climate policies using the Greenhouse Gas and Air Pollution Interactions and Synergies (GAINS) model in the time horizon up to 2050. The impact analysis is based on projections of energy consumption provided by the Prospective Outlook for the Long term Energy System (POLES) model for a scenario without any global greenhouse gas mitigation efforts, and for a 2°C climate policy scenario which assumes internationally coordinated action to mitigate climate change. Outcomes of the analysis are reported globally and for key world regions: the European Union (EU), China, India and the United States. The assessment takes into account current air pollution control legislation in each country. Expenditures on air pollution control under the global climate mitigation regime are reduced in 2050 by 250 billion € when compared to the case without climate measures. Around one third of financial co-benefits estimated world-wide in this study by 2050 occur in China, while an annual cost saving of 35 billion (Euros) € is estimated for the EU if the current air pollution legislation and climate policies are adopted in parallel. Health impacts of air pollution are quantified in terms of loss of life expectancy related to the exposure from anthropogenic emissions of fine particles, as well as in terms of premature mortality due to ground-level ozone. For example in China, current ambient concentrations of particulate matter are responsible for about 40 months-losses in the average life expectancy. In 2050, the climate strategies reduce this indicator by 50 %. Decrease of ozone concentrations estimated for the climate scenario might save nearly 20,000 cases of premature death per year. Similarly significant are reductions of impacts on ecosystems due to acidification and eutrophication.  相似文献   
185.
Numerous different bioreactor systems are applied for hydrogen production by dark fermentation. Thermophilic fermentations are gaining an increased interest due to the high hydrogen yields associated with them. In order to reach the best thermophilic fermentation system, 2 types of bioreactors, a trickling bed and a fluidized bed system, were constructed and operated under similar conditions. Both systems were designed to meet the requirements of thermophilic fermentations, such as reduction of hydrogen partial pressure, system immanence as its best as well as increasing cell densities. For comparing the 2 systems, the extreme thermophilic organism Caldicellulosiruptor owensensis OLT and a glucose-containing medium were employed. Parameters like hydraulic retention time, glucose concentration and stripping gas amount were varied. Each bioreactor system exhibited certain advantages; the trickling bed system enabled yields close to 3 mol-H2 (mol-glucose)?1 and productivities of 0.2 L L?1 h?1, but the application of stripping gas seemed to be obligatory. The fermentations in the fluidized bed system were characterized by slightly higher productivities (0.25 L L?1 h?1), but generally lower yields. However, operation of this system without stripping gas was possible.  相似文献   
186.
We have determined feasibility and levels of agreement for visualizing the nuchal translucency within a time limit with three-dimensional (3D) and two-dimensional (2D) transvaginal ultrasound. A total of 213 women of median gestational age 13 weeks (range 11–14 weeks) undergoing first-trimester nuchal translucency (NT) thickness measurements with 2D transabdominal ultrasound were included in the study. Additionally all women underwent 2D and 3D transvaginal NT examination within a time limit of 90 s. These two methods were compared with each other and with our standard method (2D transabdominal ultrasound) with respect to visualization of fetal nuchal fold and to the levels of agreement. The nuchal fold was visualized by 3D and 2D transvaginal ultrasound in 85.9% (95% CI: 80.5%, 90.3%) and 25.8% (95% CI: 20.1%, 32.2%), respectively (p<0.001). There was a statistically significant underestimation of the NT by 2D transabdominal as compared with 3D transvaginal ultrasound of 0.1 mm (p<0.001), and by 3D transvaginal as compared with 2D transvaginal ultrasound of even 0.1 mm (p<0.001). 3D transvaginal ultrasound of the nuchal fold has increased feasibility to 2D transvaginal ultrasound within a short examination time and with minimal, but significant, measurement differences. Copyright © 2001 John Wiley & Sons, Ltd.  相似文献   
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189.
The objective of this paper is to present observations, results from monitoring measurements, and preliminary conclusions about the development of patterns and structures during the first 5 years of development of an artificial catchment starting from point zero. We discuss the high relevance of initial system traits and external events for the system development and draw conclusions for further research. These investigations as part of a Collaborative Research Center, aim to disentangle and understand the feedback mechanisms and interrelationships of processes and their co-development with spatial and temporal structures and patterns by studying an initial, probably less complex ecosystem. Therefore, intensive measurements were carried out in the catchment with regard to the development of surface structures, hydrological patterns, vegetation dynamics, water chemistry, and element budgets. During the first 5 years, considerable changes within the catchment were observed. Both internal and external factors could be identified as driving forces for the formation of structures and patterns in the artificial catchment. Initial structures formed by the construction process and initial substrate characteristics were decisive for the distribution and flow of water. External factors like episodic events triggered erosion and dissection during this initial phase, promoted by the low vegetation cover, and the unconsolidated sandy substrate. The transformation of the initial geosystem into areas with evolving terrestrial or aquatic characteristics and from a very episodic to a more permanent stream network and discharge, together with the observed vegetation dynamics increased site diversity and heterogeneity with respect to water and nutrient availability and transformation processes compared with the more homogenous conditions at point zero. The processes and feedback mechanisms in the initial development of a new landscape may deviate in rates, intensity, and dominance from those known from mature ecosystems. It is therefore crucial to understand these early phases of ecosystem development and to disentangle the increasingly complex interactions between the evolving terrestrial and aquatic, biotic, and abiotic compartments of the system. Long-term monitoring of initial ecosystems may provide important data and parameters on processes and the crucial role of spatial and temporal structures and patterns to solve these problems. Artificially created catchments could be a suitable tool to study these initial developments at the landscape scale under known, designed, and defined boundary conditions.  相似文献   
190.
This paper deals with the physical nature of the fly ashes obtained from two thermal power plants, situated in West Bengal, India. The fly ash samples are characterized by using comprehensive techniques with an emphasis on their ultrafine nature. The particle sizes of the samples are estimated using scanning electron microcopy (SEM) and found to lie within 0.18–5.90 μm. For morphology and compositional analysis, we also use SEM coupled with energy dispersive X-ray spectrometry. From X-ray study of the fly ashes the nature of conglomeration is seen to be crystalline, and the major components are mullite (Al6Si2O13) and quartz (SiO2). The magnetic measurement of the fly ash samples was carried out by SQUID magnetometer. 57Fe Mössbauer spectra are obtained using a conventional constant-acceleration spectrometer with a 57Co/Rh Mössbauer source. The hyperfine parameters obtained, in general, support the findings as made from XRD analysis and provide a quantitative measure of different iron ions present in the samples. The paper presents experimental data on the physical aspects of the fly ash samples of the thermal power plants which comprise coarse, fine, and ultrafine magnetic particulate materials and attempts to provide an exhaustive analysis.  相似文献   
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