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Juberg–Hayward syndrome is a rare autosomal recessive syndrome characterised by the association of growth retardation, microcephaly, cleft lip and palate, and thumb and radial ray abnormalities. To date, no prenatal cases have been reported. Here, we report on the first prenatal case of Juberg–Hayward syndrome. The diagnosis was established following fetopathological study. Besides the cardinal features of the syndrome, this prenatal case was remarkable for the severity of the short arm malformation and by the finding of big toe agenesis and cerebral abnormalities including hydrocephalus, agenesis of corpus callosum, and cerebellar hypoplasia. We conclude that the diagnosis of Juberg–Hayward syndrome can be discussed prenatally following ultrasound diagnosis of the association of intrauterine growth restriction, microcephaly, thumb/radial anomalies, and cleft lip/palate. Copyright © 2005 John Wiley & Sons, Ltd.  相似文献   
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A study was undertaken to establish the role of free amino acids (FAA) in aerobic energy dissipation in embryos of turbot (Scophthalmus maximus) which contain an oil globule in the egg. Laboratory-reared developing eggs and larvae (15°C, 34 salinity) were measured for oxygen uptake, ammonia excretion, contents of FAA, protein, and ammonium, and volumes of yolksac and oil globule. Newly spawned eggs from different batches contained 55 to 90 nmol egg–1 of FAA. Resorption of FAA occurred in parallel with the consumption of yolk. Resorption of the oil globule, however, occurred predominantly after hatching and mainly after yolk resorption. The combined data suggest that approximately 70% of the FAA are utilized as an energy substrate, while the rest are polymerized into body proteins. FAA become a significant energy substrate in the early egg stage and account for 100% of the aerobic energy dissipation 2 d after Fertilization then decrease to ca. 60% at the time of hatching. Lipids derived from the oil globule seem to be the main fuel after hatching and account for ca. 90% of the energy dissipation at the onset of first-feeding. Thus, the energetics of fish embryos which contain an oil globule seems to be different from those that depend exclusively on the nutritional reserves of the yolk.  相似文献   
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K. Harada 《Marine Biology》1992,112(3):383-388
The present study on chemotaxis activity was conducted between May 1989 and March 1990. In order to establish a chemotaxis index, with special reference to a repellence index of food for black abalone Haliotis discus, a logistic curve y=g/{1+ exp [-r(j-a)]}, where g, r and a are constants, was applied to time-courses of abalone responses to fresh and spoiled algal extracts. The time-courses were respresented by the number of individuals (y i) displaying three behaviors (entering. leaving and remaining in a test compartment with algal sample) over time (j=1 to 10, 2-min intervals). Repellence and attraction specimens were prepared from a homogenate of green laver Ulva pertusa allowed to spoil for 2, 4 and 6 d at 40°C, and from fresh homogenate, respectively. Of 32 time-courses (estimated according to a method whereby the observed number was weighted by j for each behavior, involving 30 individuals), 32 entered, 19 left and 19 remained time-courses fitted the curve at the 0.10 level, as determined by a chi-square test. A general evaluation of repellence and attraction, determined by observable behavior, agreed well with the difference in a between the dummy (control) and specimen curves, and with the ratio of a in the dummy curve to a in the specimen curve, for logistic curves fitted to the entred time-course. The difference was >0 and the ratio was >1 in the repellence index, and <0 or <1 respectively in the attraction index. The validity of the indexes was ascertained using food specimens prepared from three kinds of algae.  相似文献   
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The possible involvement of calmodulin-dependent processes in the control of Na+ and Cl- transport pathways has been investigated on isolated, perfused preparations of salt-transporting posterior gills of the euryhaline Chinese crab Eriocheir sinensis (collected near Emden, Germany in autumn 1990). The anti-calmodulin phenothiazine drugs Chlorpromazine and Trifluoperazine induced depolarization of the transepithelial potential only when added to the serosal bathing saline (socalled in). This effect is best interpreted by assuming a disturbance of the conductive Cl- pathways located at the baso-lateral side of the epithelium. In agreement with that conclusion is the fact that Trifluoperazine inhibits the Cl- transepithelial influx. Trifluoperazine also induces inhibition of the Na+ influx when added either to the incubation (out) or to the perfusion (in) medium. These results indicate inhibitory effects of the anticalmodulin drug on both the Na+/K+ pump and leak system located at the serosal side and on the Na+/H+ exchange located at the apical side of the epithelium.  相似文献   
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Previous epidemiological studies have shown that dental fluorosis is endemic in the lowland, dry zone of Sri Lanka, which is considered to be an area in which excessive quantities of fluorides are present in the drinking water supplies. It has been found that kaolinitic clay forms a suitable raw material in the defluoridation of water.It is shown that there is a noticeable effect of selenium and media pH on the reactions involved in the interaction of fluoride with clay. In this study, 1 mM fluoride solutions containing SeO 3 2– (selenite) concentrations of 0 mM, 0.1 mM, 0.5 mM and 1 mM were used in the reactions with kaolinitic clay. The effect of pH was monitored in the range 4 to 8. It was observed that fluoride adsorption was maximum at a pH of 5.6 without either SeO 3 2– or SeO 4 2– , the adsorption capacity being 15.2 mol F g–1 clay. However, when the SeO 3 2– concentration was increased up to 0.5 mM at this optimum pH, the adsorption capacity reduced to 12.8 mol F g–1 clay. Monitoring of the effect of SeO 4 2– and media pH on fluoride adsorption showed that when the SeO 4 2– concentration increases from zero to 0.1 mM, there is a reduction of fluoride adsorption capacity. However, when the SeO 4 2– concentration is further increased from 0.1 mM to 1.0 mM, there was an increase in the fluoride adsorption capacity, indicating a more consistent effect of SeO 3 2– on fluoride-kaolinitic clay interaction than SeO 4 2– .Fluoride concentrations in drinking water supplies have a marked effect on dental health and the geochemistry of selenium appears to play an important role in the geochemical mobility of fluoride ions.  相似文献   
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Three simple steady-state water-chemistry models are used to calculate critical loads of sulfur for lakes in Finland. Because of the high concentrations of organic matter in Finnish lakes, the influence of organic anions on the calculation of critical loads has been given special attention. The first two methods are well known ion-balance methods which have been used in many previous lake-acidification studies. The third method, developed for this study, includes the numerical solution of equilibrium equations for organic anions, inorganic carbon species and inorganic monomeric aluminum. The original pH and aluminum concentration of the lakes are estimated with this model, and a method to estimate the original acid neutralizing capacity (ANC) by simulating a Gran-titration is also tested on the lake data. Uncertainty in the predictions is estimated by varying the most critical model parameters.  相似文献   
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Summary The predator,Rhizophagus grandis, is linked to its specific prey,Dendroctonus micans, by semiochemical signals regulating oviposition and long range orientation. A mixture of simple oxygenated monoterpenes [(–)—fenchone, (–)—pinocamphone, rac. camphor, terpinene-4-ol, borneol, fenchol and verbenone], identified from the frass ofD. micans has been found to be extremely active in a flight windtunnel as a long—range attractant for the predator. The mixture elicited 84 % of the response to larval frass of the prey. Excluding pinocamphone from the mixture did not influence its activity. Also, changing the absolute configurations of some of the components (fenchol, terpinene-4-ol and borneol) did not influence the predators' response. However, the addition of (–)—-terpineol increased the attractivity of the synthetic blend to almost that of larval frass of the prey. Ecological implications of the identified semiochemicals and their use in pest management are discussed.  相似文献   
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