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991.
We report the prenatal diagnosis of two fetuses with Sanfilippo disease type B. In both pregnancies there were excessive amounts of heparan sulphate in amniotic fluid and the activity of N-acetyl-α-D-glucosaminidase was undetectable in cultured amniotic fluid cells. The predictions were confirmed by enzyme assay of cultured skin fibroblasts from the aborted fetus or the affected infant. The disorder was excluded for two other pregnancies at risk and the predictions are considered to be correct because of the normal progress of the healthy children.  相似文献   
992.
The kinetics of the reaction of dissolved S(IV) with H2O2 was studied on 59 precipitation samples collected in Southwestern China, during the period of June 1988 to October 1989, from which the oxidation rates of the reaction were measured. The extent of reaction was followed by continuous amperometric measurement of the concentrations of the reagents. The two systems (i. e. rainwater and purified water) have been studied with the same methodology under identical reaction conditions (e. g. reagent concentrations, ionic strength and temperature). The kinetics was studied in no buffer solutions. The effect of formaldehyde on the reaction has also been studied, including kinetic studies in laboratory and model calculations, and it is indicated that formaldehyde with typical atmospheric concentration exerts no influence on the reaction of dissolved S(IV) with H2O2. In addition, the activation energy of the reaction was also measured in purified water as reaction medium for temperature range 0-50℃ at pH 4.0  相似文献   
993.
994.
Leukocyte adhesion deficiency (LAD) is a rare autosomal recessive disorder which leads to recurrent severe infections due to impaired leukocyte functions. The disorder is caused by an absence or deficiency of leukocyte cell adhesion molecules (LeuCAMs) on the leukocyte membranes. The diagnosis is established with monoclonal antibodies against the LeuCAMs. We have carried out a prenatal diagnostic procedure by means of cordocentesis in a mother who was 20 weeks pregnant and had previously given birth to a child with LAD. This previous child had the severe form of LAD with undetectable mRNA for the β chain, the common subunit of the LeuCAMs. We found that the fetal granulocytes expressed the LeuCAMs normally. At birth, the baby was physically normal and showed no signs of impaired leukocyte functions.  相似文献   
995.
青海省格尔木地区地下水位六年来平均上升了6.203m,造成了国内外罕见的严重灾害。本文以实际资料为依据,用水均衡方法研究地下水位上升机理。以水库蓄水为界限,划分两个均衡期,分别建立方程并求出使地下水位上升的储存量的增加量为4.84m~3/s。分解后得水库岸边渗漏量及水库调蓄、控水引发的河水渗漏量为10.51m~3/s,其储存量的增加量是4.31m~3/s,占总量的88.8%。  相似文献   
996.
两性高分子絮凝剂阴、阳离子基团含量对絮凝性能的影响   总被引:9,自引:0,他引:9  
合成了一系列带不同阴、阳离子基团含量的高分子絮凝剂,详细讨论了阴、阳离子基团含量对硅藻土悬浮体系絮凝性能(透光率、沉降速度、沉降体积)的影响。结果表明:阴、阳离子基团含量并不是越大越好,它们之间有个协同效应,基团含量太大或太小对絮凝作用都不利。  相似文献   
997.
6   ha, or 6% of the country's total area. The environmental implications of this large-scale conversion have so far received little attention; the present study examined some of these implications. A review of research on soil physical and chemical aspects of the conversion of permanent grassland into arable land reveals that such a large-scale conversion may have considerable effects upon the environment. For example, due to the mineralization of soil organic matter a release of NO3 and CO2 into the environment can be expected on the order of 10 t N and 100 t C per hectare. Environmentally equally severe, if not worse, is the increased amount of surface runoff that can be expected from converted grassland soils in arable land during winter because of surface sealing and soil compaction. This increased surface runoff, in combination with the runoff from other farmland, may be one of the reasons for the growing frequency of floods along major German rivers in recent years. In view of the lasting adverse environmental effects of permanent grassland conversion and the subsidized agricultural surpluses in Germany today, we conclude that a reconversion of arable land into permanent grassland may be beneficial both environmentally and economically.  相似文献   
998.
分布在世界各地的K/T界面剖面,尽管形成环境各异,但彼此之间的岩石矿物特征却有许多共同之处。对于大多数剖面来说,界面粘土层的底都普遍存在着一个结构特别的冲击层。冲击层的厚度在全球的分布并不均匀,北美和加勒比海地区可达2~50cm,而其它地区则只有几厘米。冲击层的厚度可能反映了距离撞击坑的远近。详细的岩石与矿物学研究结果表明,在北美和加勒比海地区冲击层实际上具有双层结构特征。上面的一层称为火球层,除了富集Ir和其它地外指示元素外,还含有大量的烟灰和富Ni镁铁尖晶石。下面的溅射层则含有大量的冲击玻璃球粒(玻璃陨石)和冲击变质矿物。K/T界面冲击层的发现为Ir异常的地外撞击成因解释提供了更为直接的物理证据。  相似文献   
999.
Prenatal diagnosis for infantile osteopetrosis was attempted during the third pregnancy of a first-cousin marriage whose family history revealed an affected previous child. At the 25th week of pregnancy, fetal X-ray evaluation revealed marked sclerosis of osteopetrotic bone and metaphyseal splaying and clubbing of both femurs. The pregnancy was terminated and repeated X-rays and histopathological examination of fetal bone (femur) confirmed the diagnosis.  相似文献   
1000.
采用“粉未冶金法”生产Ag—CdO。该方法工艺简单,生产周期短,生产效率高。在整个生产工艺过程中,采用密闭型的设备操作,控制生产工艺过程中氧化粉尘逸出,研究出了Ag—CdO无(微)公害生产工艺。经监测,氧化镉浓度达到国家《工业企业设计卫生标准》(TJ36—79)的要求。  相似文献   
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