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Prenatal diagnosis of Sandhoff disease (infantile onset) at 16 weeks gestation has been made by detection and analysis of N-acetylglucosaminyl-oligosaccharides in amniotic fluid using high performance liquid chromatography. The elution profile for the branched chain oli-gosaccharides was identical with that obtained with neonatal and infantile Sandhoff urine. The concentration of the oligosaccharides in the fluid was 1/100th that of urine but when calculated relative to creatinine the levels were similar. No oligosaccharides were detected in normal control amniotic fluids (10 patients) at a similar gestational age. Based on the levels of the amniotic fluid oligosaccharides and the sensitivity limits of the assay, prenatal diagnosis of patients with the juvenile onset form of the disease may also be possible with this technique. 相似文献
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At 10 weeks' gestation, chorionic villus biopsy was obtained from a woman at risk for Sanfilippo type C disease. Acetyl-CoA: α-glucosaminide N-acetyltransferase activity was markedly deficient. The pregnancy was terminated at 12 weeks' gestation and follow-up study on fetal fibroblasts confirmed the diagnosis. 相似文献
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638.
A field study was conducted to evaluate the protozoan colonization patterns on artificial substrates in relation to organic pollution within a tropical harbour. The composition of protozoans and their succession rates on artificial substrates(polyurethane foam units) were compared between two field stations(A and B), and their presence were considered with regards to the prevailing water quality conditions at the study sites. Altogether 44 genera of flagellates and ciliates were documented. The common genera of flagellates encountered included Monas, Polytoma, and Chromalina. Among the ciliates, the predominant genera were Tetrahymena, Vorticella, Lagymophyra, and Heloiphyra. These groups exhibited characteristic successional patterns in relation to ambient water quality. At Station A, located close to the sewage outfall, the water quality parameters included poor Secchi-disc transparency(0.48 m), dissolved oxygen of 1.93 mg/ml, salinity of 18 psu, and temperature 31.3 degrees C. Here, the nanoflagellates (spumella) colonized first, followed by microcilliate(Tetrahymena) and sessile form(Vorticella). Station B, located on the seaward side, was characterized by relatively less-stressed environmental conditions with transparency 1.85 m and dissolved oxygen value of 6.04 mg/ml. Salinity of 27.27 psu, and mean temperature of 30 degrees C were recorded at "B". At this station, the nanoflagellate Polytoma was first documented to colonize on the substrates, followed by microcilliate(Lagynophrya) and suctorid(Heliophyra). These findings support the use of protozoans as indicator species for evaluating the hazards posed by organic pollution to natural estuarine communities. 相似文献
639.
High-performance permanent magnets 总被引:2,自引:0,他引:2
High-performance permanent magnets (pms) are based on compounds with outstanding intrinsic magnetic properties as well as
on optimized microstructures and alloy compositions. The most powerful pm materials at present are RE–TM intermetallic alloys
which derive their exceptional magnetic properties from the favourable combination of rare earth metals (RE=Nd, Pr, Sm) with
transition metals (TM=Fe, Co), in particular magnets based on (Nd,Pr)2Fe14B and Sm2(Co,Cu,Fe,Zr)17. Their development during the last 20 years has involved a dramatic improvement in their performance by a factor of >15 compared
with conventional ferrite pms therefore contributing positively to the ever-increasing demand for pms in many (including new)
application fields, to the extent that RE–TM pms now account for nearly half of the worldwide market. This review article
first gives a brief introduction to the basics of ferromagnetism to confer an insight into the variety of (permanent) magnets,
their manufacture and application fields. We then examine the rather complex relationship between the microstructure and the
magnetic properties for the two highest-performance and most promising pm materials mentioned. By using numerical micromagnetic
simulations on the basis of the Finite Element technique the correlation can be quantitatively predicted, thus providing a
powerful tool for the further development of optimized high-performance pms. 相似文献
640.
H. Kühne 《Die Naturwissenschaften》1957,44(15):431-431