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Hydrocephalus-stenosis of the acqueduct of Sylvius sequence (HSAS) is characterized by hydrocephalus, macrocephaly, adducted thumbs, spasticity, agenesis of the corpus callosum and mental retardation. X-linked hydrocephalus is known to be due to mutations in the gene coding for the neural cell adhesion molecule L1 (L1-CAM) and diagnosis is made by identification of a mutation in the L1-CAM gene. Prenatal diagnosis of HSAS is usually suggested on ultrasound examination showing hydrocephalus in a male fetus associated with bilateral adducted thumbs. Mutation screening of the L1-CAM gene is indicated when neuropathological examination shows hypoplasia of the corticospinal tract associated with aqueductal stenosis. We report here two cases of HSAS diagnosed within the same family by ultrasound examination in the first trimester of pregnancy when bilateral adducted thumbs were the only early ultrasound marker. Copyright © 2001 John Wiley & Sons, Ltd.  相似文献   
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Seventeen different wastewater sludges were characterized using both chemical and organic matter fractionation methods (water extraction, Van Soest method, and acid hydrolysis) and 6-mo incubation studies to assess their decomposition in soil. Simple correlation and multiple factor analysis (MFA) were then performed to establish relationships between composition and C and N mineralization of sludges. Carbon and N concentrations covered a wide range of values, but organic carbon (C(o)) to organic nitrogen (N(o)) ratios were relatively low (from 5 to 19). Carbon and N were mainly distributed in the most soluble fractions of the Van Soest method and in the water-insoluble fraction at 100 degrees C. Carbon mineralization varied from 180 to 661 g C kg(-1) organic C added during the 168-d incubation. The addition of sludges led to different inorganic N dynamics: from -3.3 to +120.0 g N kg(-1) sludge organic C mineralized after the 168-d incubation. Fractionation studies showed that the most discriminating method was acid hydrolysis. Carbon mineralization was linked with the proportion of sludge N and C present in the lignin-like fraction (r = -0.68 and -0.65, respectively). Significant relationships were established between N mineralization and N(o) to C(o) ratio (0.88 < r < 0.95) and the C(o) to N(o) ratio of sludges, the C to N ratio of the soluble fraction obtained by the Van Soest method, the water-soluble fraction at 100 degrees C, and the C and N present in the acid-hydrolyzable fraction. Finally, multiple factor analysis also enabled establishing a sludge typology using five clusters based on composition and mineralization characteristics.  相似文献   
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Constant environmental changes in oceanic and estuarine systems due to human activity in coastal zones require different tools and strategies for their study to be able to assess the health status of aquatic ecosystems. The Gulf of Mexico has a tremendous ecological importance because of its biological diversity. Baseline levels of blood metabolites and immune components were defined for wild tropical populations of Litopenaeus setiferus, an important shrimp species of the Gulf of Mexico. Osmotic pressure, oxy hemocyanin (OxyHc), glucose, proteins, cholesterol, lactate, and triacylglycerols (TAG) were used as indicators of physiological status; pro phenoloxydase (proPO) and phenoloxydase (PO) quantification of hemocytes, as well as respiratory burst, were used to assess the immunological status. Significant differences were observed in live weight of juveniles, males, and females of L. setiferus with mean values of 10.54, 35.05, and 40.33 g, respectively. A value of 460 mOsm/kg was recorded in the water where juveniles were sampled and 1,000 mOsm/kg in adult sampling zones. Consequently, the osmotic pressure of L. setiferus juveniles (718.02 mOsm/kg) was 22% lower than in adults (925 mOsm/kg); there were no differences between sexes. A significantly lower value in juvenile OxyHc was observed. This value was 24 and 16% lower than that observed in males and females, respectively. Hemolymph glucose, cholesterol, and triacylglycerol levels were not statistically different between shrimp stages, showing median values of 0.19, 0.32, and 0.39 mg/ml of glucose, cholesterol, and triacylglycerols, respectively. Hemocyanin was distributed normally in the juvenile–male population but not in females. A proportion between 52 to 81% of OxyHc of the total protein (OxyHc/Prot) was obtained for all stages. Digestive gland glycogen showed no normal distribution in juveniles and adults. Digestive gland glycogen was significantly higher in juveniles (median value of 2.64 mg/g) than in adults (median value of 1.58 mg/g). A higher value of lactate (0.11 mg/ml) was observed in juveniles than in males (0.09 mg/ml) and females (0.04 mg/ml). Granular cells (GC) corresponded to 36, 44, and 39% of the total hemocytes (TH) observed in juveniles, males, and females. ProPO showed no normal distribution in all stages. A high proPO activity was recorded in L. setiferus females that was 95% higher than in the juvenile–male group. Respiratory burst was divided into two groups, juveniles–males and females, and was distributed normally in both. Female respiratory burst values were 40 and 45% higher than those observed in the juvenile and male groups, respectively. There was an increment in OxyHc, proteins, PO, hemocytes, and respiratory burst with an increase in body weight. In contrast, the relationship of live weight to glucose, TAG, and proPO revealed no significant regressions. A significant decrease in cholesterol, glycogen, and lactate with an increase in body weight was also observed. We now have indicators of nutritional and immune status of juveniles and adults that can reveal changes in trophic relations and health at the population level. This will serve as a basis for using L. setiferus as environmental sentinels, as non-controversial and inexpensive models to evaluate the potential changes that human activities could exert on this important marine ecosystem.Communicated by P.W. Sammarco, Chauvin  相似文献   
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ABSTRACT: The U.S. Geological Survey examined 25 agricultural streams in eastern Wisconsin the determine relations between fish, invertebrate, and algal metrics and multiple spatial scales of land cover, geologic setting, hydrologic, aquatic habitat, and water chemistry data. Spearman correlation and redundancy analyses were used to examine relations among biotic metrics and environmental characteristics. Riparian vegetation, geologic, and hydrologic conditions affected the response of biotic metrics to watershed agricultural land cover but the relations were aquatic assemblage dependent. It was difficult to separate the interrelated effects of geologic setting, watershed and buffer land cover, and base flow. Watershed and buffer land cover, geologic setting, reach riparian vegetation width, and stream size affected the fish IBI, invertebrate diversity, diatom IBI, and number of algal taxa; however, the invertebrate FBI, percentage of EPT, and the diatom pollution index were more influenced by nutrient concentrations and flow variability. Fish IBI scores seemed most sensitive to land cover in the entire stream network buffer, more so than watershed‐scale land cover and segment or reach riparian vegetation width. All but one stream with more than approximately 10 percent buffer agriculture had fish IBI scores of fair or poor. In general, the invertebrate and algal metrics used in this study were not as sensitive to land cover effects as fish metrics. Some of the reach‐scale characteristics, such as width/depth ratios, velocity, and bank stability, could be related to watershed influences of both land cover and geologic setting. The Wisconsin habitat index was related to watershed geologic setting, watershed and buffer land cover, riparian vegetation width, and base flow, and appeared to be a good indicator of stream quality Results from this study emphasize the value of using more than one or two biotic metrics to assess water quality and the importance of environmental characteristics at multiple scales.  相似文献   
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Facultative joint colony founding by social insects provides opportunities to analyze the roles of genetic and ecological factors in the evolution of cooperation. Although cooperative nesting is observed in range of social insect taxa, the most detailed studies of this behavior have been conducted with Hymenoptera (ants, bees, and wasps). Here, we show that foundress associations in the haplodiploid social thrips Dunatothrips aneurae (Insecta: Thysanoptera) are most often comprised of close relatives (sisters), though groups with unrelated foundresses are also found. Associations among relatives appear to be facilitated by limited female dispersal, which results in viscous population structure. In addition, we found that per capita productivity declined with increasing group size, sex ratios were female-biased, and some female offspring apparently remained in their natal domicile for some time following eclosion. D. aneurae thus exhibits a suite of similarities with eusocial Hymenoptera, providing evidence for the convergent evolution of associated social and life-history traits in Hymenoptera and Thysanoptera.  相似文献   
140.
Hugueny B  Cornell HV  Harrison S 《Ecology》2007,88(7):1696-1706
Many natural communities exhibit positive relationships between local and regional species richness (LSR-RSR relationships), which can be either linear or curvilinear. Previous models have shown that the form of this relationship depends on the relative rates of colonization and extinction and the sensitivity of these rates to competition. We use simple models to show that the LSR-RSR relationship also depends on the type of metacommunity structure (Levins-like or mainland-island), and our models generate a wider range of realistic forms than do most previous models. We parameterize and test our models with two independent data sets for Daphnia in rock pools on islands in Finland and Sweden. We find that the Levins-like model with competition correctly predicts the observed LSR-RSR relationship and provides the best fit to the average local species richness per island. Simulations show that our models are robust to relaxing our assumption of identical species properties. Our study is one of the first to make and successfully test quantitative predictions for how a widely studied community pattern, the LSR-RSR relationship, arises from metacommunity dynamics.  相似文献   
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