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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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An icebreaker cruise into the Beaufort Sea in the fall of 1986 provided a unique opportunity for studying planktonic bioluminescence in ice fields and in the marginal ice zone. Bathyphotometer casts (bioluminescence intensity, seawater temperature, beam attenuation coefficient, and salinity) and biological collections were made to a depth of 100 m. A light budget, which describes the planktonic species responsible for the measured bioluminescence, and a dinoflagellate species budget were constructed from the mean light output from luminescent plankton and plankton counts. The vertical distribution of bioluminescence among the ice stations was similar. The maximum intensities were 2 to 8×106 photons s-1 cm-3 in the upper 50 m of the sea-ice interface. The marginal ice zone station (MIZ) exhibited a maximum intensity of 2 to 3×108 photons s-1 cm-3 between 5 and 30 m depth. At Ice Station 2, Metridia longa and their nauplii contributed approximately 80% of stimulable bioluminescence in the upper 10 m but, overall, Protoperidinium spp. dinoflagellates contributed most of the light to a depth of 100 m. In the MIZ, Protoperidinium spp. dinoflagellates contributed 90% of the light within the upper 10 m, decreasing to 43% of the contributed light at a depth of 40 m. Below 40 m, dinoflagellate bioluminescence decreased to a few percent of the total to a depth of 90 m. Metridia spp. copepods contributed more than 50% of the light at depths from 40 to 90 m. Ostracods, larvaceans, and euphausiid furcilia contributed <1% of all bioluminescence at all depths sampled. Correlation analyses between measured bioluminescence (photons s-1 cm-3), the number of bioluminescent dinoflagellates and the light budget for the MIZ indicated highly significant associations: r=0.919, p=0.001, and r=0.912, p<0.001, respectively (Student's two-tailed t-tests). Bioluminescence was negatively correlated with seawater salinity at all stations (p=0.001). Maximum bioluminescence was measured in the less saline surface waters at all stations.  相似文献   
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The abundance and biomass of marine planktonic ciliates were determined at monthly intervals at two stations in Southampton Water between June 1986 and June 1987. The two stations, an outer one at Calshot and an inner one at N. W. Netley, were subject to differing marine and terrestrial influences. The potential ciliate production at cach station on each visit was estimated from these data. Enumeration of ciliates and measurements of biovolume were performed on Lugol's iodinepreserved samples and potential production was calculated using a predictive relationship based on temperature and cell volume. Heterotrophic ciliate abundance and biomass were greatest at both stations during spring and summer months, with respective maxima of 16x103 organisms 1-1 and 219 g Cl-1 recorded at N. W. Netley. Estimates of the potential production of the ciliate community ranged from <1 to 18 g Cl-1 d-1 at Calshot and <1 to 141 g Cl-1 at N. W. Netley, with annual values of 2 and 9 mg Cl-1 yr-1, respectively. Abundances, biomass and potential production estimates were generally greater at N. W. Netley than at Calshot. Carbon flow through the ciliate community was assessed using annual production values from both this study and the literature. The annual ciliate carbon requirement was equivalent to 9 and 11% of annual primary production at Calshot and N. W. Netley, and potential annual ciliate production was equivalent to 34% and >100% of the energy requirements of metazoan zooplankton at these locations, although comprising only 8 and 10% of their available food.  相似文献   
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The concept of intrinsic value and transgenic animals   总被引:1,自引:0,他引:1  
The creation of transgenic animals by means of modern techniques of genetic manipulation is evaluated in the light of different interpretations of the concept of intrinsic value. The zoocentric interpretation, emphasizing the suffering of individual, sentient animals, is described as an extension of the anthropocentric interpretation. In a biocentric or ecocentric approach the concept of intrinsic value first of all denotes independence of humans and a non-instrumental relation to animals. In the zoocentric approach of Bernard Rollin, genetic engineering is seen as a morally neutral tool, as long as the animal does not suffer as a result of it. Robert Colwell who defends an ecocentric ethic, makes a sharp distinction between wild animals and domesticated animals. Genetic manipulation of wild species is a serious moral issue, in contrast to genetic manipulation of domesticated species which is no problem at all for Colwell. Both authors do not take the species-specific nature (or telos) of domesticated animals seriously. When domestication is seen as a process between the two poles of the wild animal and the human construct (which can be patented), the technique of genetic manipulation can only be seen as a further encroachment upon the intrinsic value of animals. At the level of molecular biology, the concept of an animal's telos loses its meaning.  相似文献   
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H. Sudo  M. Azeta 《Marine Biology》1992,113(2):211-217
Ontogenetic change of habitat depths of Nautilus pompilius in the Philippines (Tañon Strait) and Fiji is considered by comparing 18O/16O ratios in septa and cameral fluid of live-caught specimens and ambient sea-water. 18O values of cameral liquid become heavier with decreasing volume within a chamber, which may be due to isotopic fractionation during discharge across the siphuncular wall. All of the seven Philippine and Fiji specimens analyzed show a distinct change in 18O from light values in the first seven septa to heavier values in the succeeding septa. Two different isotopic temperature scales are obtained for the Fiji and Philippine populations, suggesting a differential vital effect of metabolism between them. Sightly light 18O values in Septa 1 to 7 and hatching at relatively high temperatures in aquaria both suggest that N. pompilius hatch at the shallowest depths within their inhabitable vertical range. Depths of postembryonic animals in the Philippines and Fiji waters estimated from the isotopic temperature-depth diagrams range from 120 to 160 m and from 440 to 520 m, respectively, both of which correlate well with capture records. More than several tens of small-scale 18O cycles are detected in the sequence of nacreous layers within the single septum of a submature Philippine specimen. This can be interpreted as reflecting daily vertical migration.  相似文献   
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