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Stickler syndrome or hereditary progressive arthro-ophthalmopathy, is an autosomal dominant condition characterized by ocular manifestations, arthritic changes, orofacial features and deafness, in variable degrees. We report the first case of prenatal diagnosis of Stickler syndrome in a child with a Pierre–Robin sequence (PRS) causing a polyhydramnios. When isolated polyhydramnios is not explained by immunological, metabolic or infectious causes, swallowing difficulty due to PRS must be considered. As PRS is aetiologically heterogenous, the prognosis depends on the cause. Genetic investigations and familial history must be taken into account. Here, in a context of familial Stickler syndrome, making the prenatal diagnosis of PRS as part of Stickler syndrome allowed us to reassure the parents and to anticipate airway trouble at the child's birth. Copyright © 2002 John Wiley & Sons, Ltd. 相似文献
554.
Host plant odours attract gravid insect females for oviposition. The identification of these plant volatile compounds is essential
for our understanding of plant–insect relationships and contributes to plant breeding for improved resistance against insects.
Chemical analysis of grape headspace and subsequent behavioural studies in the wind tunnel show that host finding in grapevine
moth Lobesia botrana is encoded by a ratio-specific blend of three ubiquitous plant volatiles. The odour signal that attracts mated females to
grape consists of the terpenoids (E)-β-caryophyllene, (E)-β-farnesene and (E)-4,8-dimethyl-1,3,7-nonatriene. These compounds represent only a fraction of the volatiles released by grapes, and they are
widespread compounds known throughout the plant kingdom. Specificity may be achieved by the blend ratio, which was 100:78:9
in grape headspace. This blend elicited anemotactic behaviour in moths at remarkably small amounts. Females were attracted
at release rates of only a few nanograms per minute, at levels nearly as low as those known for the attraction of male moths
to the female sex pheromones. 相似文献
555.
Julien Saada Olivier Parant Sylvie Kessler Jacqueline Aziza Marie France Sarramon 《黑龙江环境通报》2006,26(1):9-10
Congenital splenic cyst (CSC) is a rare condition, and only a few cases with prenatal diagnosis and no associated malformation have been reported. Spontaneous regression is possible in case of mild CSC (under 40 mm), but enlargement or secondary complications may lead to surgical treatment. We report, herein, two cases of isolated mild CSC with complete spontaneous postnatal regression. Copyright © 2006 John Wiley & Sons, Ltd. 相似文献
556.
Guo-Liang Shi Ying-Ze Tian Tong M Dan-Lin Song Lai-Dong Zhou Bo Han Yin-Chang Feng Armistead G. Russell 《环境科学学报(英文版)》2017,29(6):1-11
Long-term and synchronous monitoring of PM10 and PM2.5 was conducted in Chengdu in China from 2007 to 2013. The levels, variations, compositions and size distributions were investigated. The sources were quantified by two-way and three-way receptor models (PMF2, ME2-2way and ME2-3way). Consistent results were found: the primary source categories contributed 63.4% (PMF2), 64.8% (ME2-2way) and 66.8% (ME2-3way) to PM10, and contributed 60.9% (PMF2), 65.5% (ME2-2way) and 61.0% (ME2-3way) to PM2.5. Secondary sources contributed 31.8% (PMF2), 32.9% (ME2-2way) and 31.7% (ME2-3way) to PM10, and 35.0% (PMF2), 33.8% (ME2-2way) and 36.0% (ME2-3way) to PM2.5. The size distribution of source categories was estimated better by the ME2-3way method. The three-way model can simultaneously consider chemical species, temporal variability and PM sizes, while a two-way model independently computes datasets of different sizes. A method called source directional apportionment (SDA) was employed to quantify the contributions from various directions for each source category. Crustal dust from east-north-east (ENE) contributed the highest to both PM10 (12.7%) and PM2.5 (9.7%) in Chengdu, followed by the crustal dust from south-east (SE) for PM10 (9.8%) and secondary nitrate & secondary organic carbon from ENE for PM2.5 (9.6%). Source contributions from different directions are associated with meteorological conditions, source locations and emission patterns during the sampling period. These findings and methods provide useful tools to better understand PM pollution status and to develop effective pollution control strategies. 相似文献
557.
In this diary study, we investigated multi‐level predictors of daily counterproductive work behavior (CWB) relying on the theoretical frameworks of affective events theory and the emotion‐centered model of CWB. We assessed significant work events, event‐based fairness perceptions, negative emotional reactions to work events, and employee CWB over a 10‐day period. We tested within‐person relations predicting CWB, and cross‐level moderating effects of two emotion regulation strategies (suppression and reappraisal). Results from a multi‐level path analysis revealed that significant work events had both direct and indirect effects on negative emotional reactions. Further, negative emotional reactions in turn mediated the relationships between significant work events and all forms of daily CWB as well as the relationship between event‐based fairness perceptions and daily CWB‐O. Results also supported the moderating role of reappraisal emotion regulation strategy on relations between significant work events and negative emotional reactions. Less support, however, was found for the moderating influence of suppression on the link between negative emotional reactions and CWB. Among the broad work event categories we identified, our supplemental analyses revealed that negative work events involving interactions with supervisors elicited the highest levels of employee negative emotional reactions. We discuss the theoretical and practical implications of these findings. Copyright © 2014 John Wiley & Sons, Ltd. 相似文献
558.
Organizational justice researchers tend to treat as synonyms the terms “justice” and “fairness”. We discuss different definitional arguments, concluding that these two concepts are distinct. Justice should be defined as adherence to rules of conduct, whereas fairness should be defined as individuals' moral evaluations of this conduct. Copyright © 2014 John Wiley & Sons, Ltd. 相似文献
559.
Mark Green Hampden Kuhns Marc Pitchford Russell Dietz Lowell Ashbaugh Tom Watson 《Journal of the Air & Waste Management Association (1995)》2013,63(5):586-595
Abstract A simple data analysis method called the Tracer-Aerosol Gradient Interpretive Technique (TAGIT) is used to attribute particulate S and SO2 at Big Bend National Park in Texas and nearby areas to local and regional sources. Particulate S at Big Bend is of concern because of its effects on atmospheric visibility. The analysis used particulate S, SO2 , and perfluorocarbon tracer data from six 6-hr sampling sites in and near Big Bend National Park. The data were collected in support of the Big Bend Regional Aerosol and Visibility Observational (BRAVO) Study; the field portion was conducted from July through October 1999. Perfluorocarbon tracer was released continuously from a tower at Eagle Pass, TX, approximately 25 km northeast of two large coal-fired power plants (Carbon I and II) in Coahuila, Mexico, and approximately 270 km east-southeast of Big Bend National Park. The perfluorocarbon tracer did not properly represent the location of the emissions from the Carbon power plants for individual 6-hr sampling periods and attributed only 3% of the particulate S and 27% of the SO2 at the 6-hr sites in and near Big Bend to sources represented by the tracer. An alternative approach using SO2 to tag “local” sources such as the Carbon plants attributed 10% of the particulate S and 75% of the SO2 at the 6-hr sites to local sources. Based on these two approaches, most of the regional (65–86%) and a small fraction (19–31%) of the local SO2 was converted to particulate S. The analysis implies that substantial reductions in particulate S at Big Bend National Park cannot be achieved by only reducing emissions from the Carbon power plants; reduction of emissions from many sources over a regional area would be necessary. 相似文献
560.
Katherine S. Wade Amit Marmur Armistead G. Russell Ben Hartsell Eric Edgerton 《Journal of the Air & Waste Management Association (1995)》2013,63(6):876-888
Abstract Data from the U.S. Environmental Protection Agency Air Quality System, the Southeastern Aerosol Research and Characterization database, and the Assessment of Spatial Aerosol Composition in Atlanta database for 1999 through 2002 have been used to characterize error associated with instrument precision and spatial variability on the assessment of the temporal variation of ambient air pollution in Atlanta, GA. These data are being used in time series epidemiologic studies in which associations of acute respiratory and cardiovascular health outcomes and daily ambient air pollutant levels are assessed. Modified semivariograms are used to quantify the effects of instrument precision and spatial variability on the assessment of daily metrics of ambient gaseous pollutants (SO2, CO, NOx, and O3) and fine particulate matter ([PM2.5] PM2.5 mass, sulfate, nitrate, ammonium, elemental carbon [EC], and organic carbon [OC]). Variation because of instrument imprecision represented 7–40% of the temporal variation in the daily pollutant measures and was largest for the PM2.5 EC and OC. Spatial variability was greatest for primary pollutants (SO2, CO, NOx, and EC). Population–weighted variation in daily ambient air pollutant levels because of both instrument imprecision and spatial variability ranged from 20% of the temporal variation for O3 to 70% of the temporal variation for SO2 and EC. Wind rose plots, corrected for diurnal and seasonal pattern effects, are used to demonstrate the impacts of local sources on monitoring station data. The results presented are being used to quantify the impacts of instrument precision and spatial variability on the assessment of health effects of ambient air pollution in Atlanta and are relevant to the interpretation of results from time series health studies that use data from fixed monitors. 相似文献