Dandy – Walker syndrome and corpus callosum agenesis in 5p deletion

5p deletion syndrome commonly known as cri du chat is well described in affected neonates with catlike cry and hypotonia. Karyotyping will usually show a deletion of the short arm of one chromosome 5 with variable breakpoints. Only a few cases have been reported prenatally, and the fetal form of the syndrome has not been clearly individualised. We report a new case of 5p deletion syndrome diagnosed prenatally in association with Dandy–Walker syndrome and agenesis of the corpus callosum. Other brain anomalies have been reported previously, but this unusual association suggests the use of a specific probe in the investigation of these malformations. Copyright © 2005 John Wiley & Sons, Ltd.     

超高速瞬态测试系统软硬件构架设计研究

目的研究采样频率为2~10 MHz的64通道超高速同步瞬态测试系统的设计技术,实现两类典型超高速瞬态测试系统的硬件架构设计与软件架构设计。方法一类采用PXI-Express高带宽总线和高速RAID磁盘阵列架构构建持续流盘存储的连续高速测试系统,另一类是采用大容量板载数据缓存和PXI总线事后下载传输数据的架构构造高速测试系统。在高性能测试软件设计方面,主要应用生产者/消费者结构与有限状态机相结合的软件架构进行高性能测试系统软件设计。结果目前64通道下基于持续流盘架构的测试系统受数据记录的速度限制系统最高采样频率仅达2.5 MHz,而基于板载缓存数据与PXI总线事后下载数据架构的测试系统最高采样频率可达10 MHz,测试时长可达5 s。结论当前两类架构的测试系统均可满足超高速瞬态测试需求,设计时需根据需求的最高采样频率决定使用的架构形式。     

外源亚硒酸盐和硒酸盐在土壤中的价态转化及其生物有效性

采用土培盆栽试验和化学分析方法相结合的方法,研究了外源亚硒酸盐和硒酸盐态硒在土壤中的价态转化及其生物有效性,旨在为富硒蔬菜的开发和硒污染土壤的生物修复提供依据.结果表明,当土壤中外源施入亚硒酸盐时,小白菜不同生长时期土壤总硒、Se(Ⅳ)和Se(0)含量均随外源硒浓度的增加显著升高(p<0.05);而当土壤外源施入硒酸盐...     

论经济欠发达地区如何建设生态工业园区——以千里山工业园区为例

资源和环境问题已成为各界关注的焦点,工业园区向现代生态工业因区建设转变是新形势生态文明下的必然要求。文章以内蒙古乌海市海勃湾区千里山工业园区为例,阐述了该工业园区建立生态型工业园区的可行性。     

基于PANDA平台的飞行器脉动压力激励随机振动响应分析

目的 形成自主再入飞行数值模拟预测技术.方法 采用模态叠加法开展自由结构的多点脉动压力激励随机振动响应分析,基于PANDA高性能力学分析平台进行并行实现研究,构建相应的求解模块.针对飞行器再入过程,基于自主研发的软件模块,分析飞行器自由状态的模态特性及其在实测脉动压力载荷下的振动响应,并与商业软件分析结果 进行比对.结...     

Prenatal diagnosis of primary anophthalmia with a 3q27 interstitial deletion involving SOX2

We report an interstitial deletion of chromosome 3q26-q28 in a fetus in which anophthalmia had been detected prenatally. FISH analysis, using BAC clones encompassing the SOX2 locus, showed that SOX2 gene was involved in the chromosomal breakpoint of the deletion. This case confirms that haploinsufficiency for SOX2 plays a crucial role in human eye development and emphasizes the necessity of careful chromosomal analysis, including FISH analysis of the 3q region, in case of prenatal discovery of anophthalmia. Copyright © 2004 John Wiley & Sons, Ltd.     

利用SPAMS研究华北乡村站点(曲周)夏季大气单颗粒物老化与混合状态

利用单颗粒气溶胶飞行时间质谱(SPAMS)于2013年6月30日~7月8日对华北地区乡村站点曲周大气单颗粒粒径及其化学组成进行了在线测量,共采集到同时含有正负离子谱图的颗粒230 152个,其粒径主要集中在0.2~2.0μm.结果表明,该地区的大气颗粒物主要分为8类:元素碳(EC,55.5%)、有机碳(OC,10.7%)、钠,钾等碱金属颗粒(alkalis,17.4%)、其他金属颗粒(other metals,1.7%)、富铁颗粒(Fe-rich,6.3%)、富铅颗粒物(Pb-rich,3.1%)、沙尘颗粒(dust,4.8%),other颗粒(0.8%),观测得到的8类气溶胶颗粒中绝大部分包含46NO-2、62NO-3、80SO-3、96SO-4、97HSO-4等二次组分离子,说明这些颗粒都经历了不同的老化过程或与二次组分进行了不同程度的混合.从气溶胶类型的谱分布看,各类型颗粒数浓度峰值基本出现在700~800 nm之间,dust、Fe颗粒主要集中在粗粒径段,EC颗粒子类研究表明随着表面不断吸附NH+4、NO-3、SO2-4等二次组分,EC颗粒逐步演化成老化程度较低的NO-3吸附型EC(ECN)和严重老化的SO2-4吸附型EC(ECS)混合态,两者日变化呈现明显的负相关性,也可能随着二次有机物在EC表面吸附,形成OC/EC混合态.     

我国废钢铁加工设备生产使用现状及前景展望

对我国2009年粗钢产量及废钢铁加工供应情况进行概述和预测,介绍了我国废钢铁加工设备的生产规模、使用状况及研发现状,以及我国废钢铁加工设备的种类及其性能。展望了我国废钢铁加工设备的前景,指出质优价廉、适合中国国情、具有环保功能的废钢铁加工设备市场发展空间巨大。     

Eutrophication development and its key regulating factors in a water-supply reservoir in North China

Yanghe Reservoir is an important source of drinking water for Qinhuangdao City,North China;however,in recent decades this water source has been eutrophic with recurrent summer cyanobacterial blooms.The trophic grade of the system in summer was mesotrophiceutrophic in 1990 and became hypertrophic in 2011.The nutrient availability is extremely high during the entire year,and the water temperature should be the primary driver of the summer blooms.In May-October of 2010 and 2011,abrupt variations were observed in the Secchi depth(SD) and chlorophyll a(Chl-a),and both the correlated analysis of Chl-a-SD and trophic status indices(TSI) deviation(TSI Chl-a-TSI SD) showed that algal cell density dominated light attenuation.During the algal bloom outbreak,the microcystin concentration was found to vary between 0.35-2.12 μg/L in 2010 and 0.11-1.86 μg/L in 2011.The maximum microcystin content was more than two times the safety limit required for drinking water.Inflow discharges were most concentrated in the summer,with periods of lower residence time and the largest water level fluctuation over the entire year.When a high availability of nutrients promoted a high Chl-a concentration in the whole system,it appeared that the instability caused by the decrease in residence time could not produce effective changes in the cyanobacterial abundance.The results indicated that nutrient enrichment in the aquatic systems of Yanghe Reservoir is the most serious problem and that the status would not been modified effectively by increasing hydrological fluctuations(e.g.,decreasing the residence time).Therefore,decreasing the nutrient concentrations is the only route to improve the water quality of this reservoir.