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121.
Ninety-two families with spinal muscular atrophy (SMA) applied for genetic counselling and further prenatal diagnosis. To minimize expenses, only one tightly linked informative marker was determined in the course of preliminary examination, and non-radioactive allele detection was preferably used. Four prenatal diagnoses of SMA type I, four of SMA type II, and one of SMA type III were made. This trial programme shows the considerable requirements, importance, and potential effectiveness of prenatal prediction of SMA in Russia.  相似文献   
122.
The diagnosis of Freeman–Sheldon syndrome was made by ultrasonographic evaluation of a 20-week fetus with a positive family history. The ultrasonographic features were abnormalities of the extremities and mouth.  相似文献   
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124.
In Acromyrmex octospinosus leaf-cutting ants the metapleural glands produce an array of antibiotic compounds that serve as a general defence against unwanted microbes on the cuticle. Leaf-cutting ants also grow mutualistic Pseudonocardiaceae bacteria on their cuticle that produce antibiotics controlling a microfungal parasite of their fungus gardens. Interaction between this bacterium and gland secretion therefore seems unavoidable. We document the typical development of bacterial growth on the cuticle of young major workers, show that growth starts a few days after eclosion, and that the maximal cover is reached after 2–3 weeks and gradually declines when workers mature. Experimental closure of the metapleural glands had no effect on the initial exponential growth phase of the bacterium, but significantly reduced the cover during the decline phase. The age-dependent abundance of the bacterium and its partial dependence on metapleural gland secretion support the hypothesis that the abundance of this mutualist is actively regulated.  相似文献   
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126.
With the advent of more stringent controls on wastewater treatment, sewage sludge production in Europe and many parts of the world is increasing. With this increase comes the problem of sludge disposal, and recycling to land arguably offers an economically and environmentally sustainable option. However, a major limitation of sewage sludge reuse is the potential release of heavy metals from the sludge and heavy metal accumulation to toxic levels in topsoils. The properties of the sludge play a crucial role in determining the initial release and subsequent availability of heavy metals in amended soils. Bioavailable forms of heavy metals in recently amended soils are most likely to be those that are bioavailable in the sewage sludge. In this paper, published research on the importance of sewage sludge characteristics on metal release and bioavailability will be reviewed and contrasted with original research. A selection of sludges from around Australia has been collected for this purpose. Through the use of incubation studies, isotope dilution techniques, ion-selective electrode measurements and 13C-NMR spectroscopy, the importance of a range of sludge properties on heavy metal behaviour in sludges and sludge-amended soils is addressed.  相似文献   
127.
 In several branches of science and technology a gaseous phase is dispersed into a liquid in the form of bubbles, a gaseous component then dissolves into the liquid and subsequently undergoes chemical reaction. The overall process performance can be improved substantially when the area of gas–liquid contact is increased. By subjecting the liquid phase to low frequency vibrations, the bubbles are shown to suffer significant breakage, induced by resonance. When the vibration is properly tuned, the interfacial area is found to increase by a factor of 1.8–2.4, depending on the properties of the liquid. Resonance-induced bubble breakage phenomena have a great potential for improving the rates of chemical processes involving fast reactions, with minimal energy input. Received: 7 July 2000 / Accepted in revised form: 28 August 2000  相似文献   
128.
Urban flooding is often used as an illustration of the potentially adverse effects of greenhouse-induced climate change on extreme events. There is however, a paucity of studies that convert climate scenarios into changes in flood damage. This account summarises the use of modelling techniques, for three flood prone urban catchments in south eastern Australia, to assess changes to urban flood losses for the 'most wet' and 'most dry' scenarios for the year 2070. The most wet scenario indicates that annual average flood damage could increase within the range of 2.5 to 10 times, under the most dry scenario flood regimes would be similar to those experienced at present. The socio-economic scenarios based on the changes to flood losses are used to consider policy responses. It is unlikely that many local government authorities will respond because of lack of interest and because of major changes to the climate scenarios proposed over the last decade. Any response is likely to be incremental and accord with the 'no regrets' and the precautionary principle'.  相似文献   
129.
Stratigraphy, lithology and depositional structures of Liassic red limestone-breccias of the Adnet Formation, including the 'Adnet Scheck', were studied at several outcrops of the Northern Calcareous Alps (NCA) south-east of Salzburg. A four-fold lithostratigraphic division is proposed for the Adnet Formation of the Osterhorn Mountains: the hemipelagic Schmiedwirt (Sinemurian) and Kehlbach (Carixian) members are separated from the pelagic Saubach Member (Toarcian) by a layer of amalgamated breccias (Scheck Member, probably Domerian to early Toarcian). Several other breccia beds occur locally from the base of the Kehlbach Member up to the lower Saubach Member. Although the sediments overlying the Scheck Member breccias are of coeval age, the ages of the underlying strata are very different. This can be explained by submarine Liassic erosion during a period of resedimentation from the middle Carixian until the early Toarcian. At least 10–15?m of partly lithified sediments were eroded by gravity flows. The entire Kehlbach Member and up to two-thirds of the Schmiedwirt Member were removed at Adnet. The breccias originated from submarine debris flows. Repeated flows over a long period and the depositional setting exclude a triggering by sea-level fluctuations. Most probably they arose from tectonically triggered slumps and slides of superficial sediments. The 'Scheck' was initiated on the steep upper slope of the drowned Triassic Adnet reef and flowed to the north-east.The Pliensbachian to early Toarcian period of tectonic activity indicated by the breccias was the most important during the Liassic in the Osterhorn Mountains and other parts of the NCA. From the large-scale regional distribution of the breccias and in accord with published data, a roughly north-east trending strike-slip fault zone is proposed, crossing the NCA south of the Osterhorn block, with a peaking activity during the Pliensbachian to early Toarcian as the cause of the tectonic movements.  相似文献   
130.
Single zircons from two orthogneiss complexes, the Grey Gneiss and Red Gneiss, the lowermost tectonic units in the Erzgebirge, were dated. The grey Freiberg Gneiss is of igneous origin and has a 207Pb/206Pb emplacement age of 550±7 Ma. A quartz monzonite from Lauenstein contains idiomorphic zircons with a mean 207Pb/206Pb age of 555±7 Ma as well as xenocrysts ranging in age between 850 and 1910 Ma. Red gneisses from the central Erzgebirge contain complex zircon populations, including numerous xenocrysts up to 2464 Ma in age. The youngest, idiomorphic, zircons in all samples yielded uniform 207Pb/206Pb ages between 550±9 and 554±10 Ma. Nd isotopic data support the interpretation of crustal anatexis for the origin of both units. Nd(t) values for the grey gneisses are –7.5 and –6.0 respectively, (mean crustal residence ages of 1.7–1.8 Ga). The red gneisses have a wider range in Nd(t) values from –7.7 to –2.8 (T DM ages of 1.4–1.8 Ga). The zircon ages document a distinct late Proterozoic phase of granitoid magmatism, similar in age to granitoids in the Lusatian block farther north-east. However, Palaeozoic deformation as well as medium pressure metamorphism ( 8 kbar/600–650° C) are identical in both gneiss units and distinguish these rocks from the Lusatian granitoids. The grey and red gneisses were overthrust by units with abundant high-pressure relicts and a contrasting P-T evolution. Zircon xenocryst and Nd model ages in the range 1000–1700 Ma are similar to those in granitoid rocks of Lusatia and the West-Sudetes, and document a pre-Cadomian basement in parts of east-central Europe that, chronologically, has similarities with the Sveconorwegian domain in the Baltic Shield.  相似文献   
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