Heterogeneity in fetal akinesia deformation sequence (FADS): autopsy confirmation in three 20–21-week fetuses

Fetal akinesia deformation sequence (FADS) is a rare condition characterized by intrauterine growth retardation (IUGR), congenital limb contractures, pulmonary hypoplasia, hydramnios and craniofacial abnormalities. The present report comprises an autopsy study of three fetuses to illustrate the variable clinical manifestations and neuropathological findings. Fetus 1 had arthrogryposis and no movement on fetal ultrasound examination. Aborted at 21 weeks, the fetus showed micrognathia, bilateral joint contracture with pterygia at the elbow and axilla. Growth retardation and pulmonary hypoplasia were not major features. Neuropathologic examination revealed anterior horn cell loss and lateral corticospinal tract degeneration in spinal cord, with marked muscular atrophy. Fetus 2, 20 weeks' gestation, had fetal akinesia, nuchal thickening, left pleural effusion, and Dandy-Walker malformation on ultrasound examination. Autopsy showed low-set ears, ocular hypertelorism, cleft palate, flexion contractures with pterygia over axilla, elbow and groin, pulmonary hypoplasia, Dandy-Walker malformation, unremarkable spinal cord and skeletal muscle. Fetus 3, 21 weeks' gestation, was aborted for fetal akinesia, neck and limb webbing and severe arthrogryposis. At autopsy, similar facial abnormalities, contracture and pterygia in neck and multiple major joints were found. Borderline pulmonary hypoplasia and severe lumbar scoliosis were also present. The brain, spinal cord and muscle were unremarkable. In these three fetuses, the prenatal ultrasound and autopsy findings were characteristic of FADS. Neurogenic spinal muscular atrophy was the basis of fetal akinesia in Case 1. Dandy-Walker malformation was present in Case 2, but the pathogenetic mechanism of fetal akinesia was not clear as spinal cord and muscle histology appeared normal. The etiology of akinesia was undetermined in Case 3; no extrinsic or intrinsic cause was identified. Copyright © 2002 John Wiley & Sons, Ltd.     

Population dynamics of the bank vole as studied using sequence functions

Sequence functions were used to construct a simulation model of the long-term population dynamics of the bank vole in Karelia. The mechanisms of population reproduction control affect the population size if it is greater than 1 and 4 ind./100 trap-days in spring and autumn, respectively.     

耐汞好氧反硝化菌的鉴定及适应汞离子的特性研究

从实验室保存的高效好氧反硝化菌种中筛选得到一株抗汞细菌并命名为X1,经生理生化特性和16SrRNA基因序列分析,初步鉴定该菌为恶臭假单胞菌(Pseudomonasputida).对菌株X1进行Hg2适应特性研究,结果表明,对于Hg2浓度为2、67++4、、mg·L-1的实验组,菌体分别需要被延滞12、284018、、h后进入对数期,而8mg·L-1实验组则不能进入对数期;在好氧反硝化过程中,Hg2浓度在7mg·L+-1范围内各实验组的好氧反硝化过程中NO3-N浓度变化速率、NO2-N累积峰值、pH特征点出现时刻随着Hg2浓度的增大而增大(延迟),而Hg2浓度呈现出同硝氮一致的下降趋势,并且在对数期内除汞率能达到100%.研究表明,菌株X1对Hg2最大适宜耐受浓度为7mg·L+-1,相应适应时间约为40h.在最大耐受浓度范围内,菌株X1的生长和好氧反硝化过程呈现出"被抑制-适应-受刺激"的变化规律,其中,被抑制的时间和受刺激的程度都随着Hg2浓度的增大而增大,主要表现为延滞期的延长和对数期的缩短.此外,在对数期,菌株X1的生长速率、达到稳定期的浓度和好+氧反硝化速率也都随着Hg2浓度的增大而增大,且大于无Hg2菌组.++     

ACP1000除气塔液体冷却器抗震分析设计

目的对除气塔液体冷却器进行抗震分析,以确保其在地震下必要的安全功能。方法以福清5/6号核电工程为背景,通过有限元软件ANSYS建立除气塔液体冷却器有限元模型,采用反应谱法进行设备的抗震分析与评定。结果通过抗震分析评定,发现设备鞍座为设计薄弱区域,故修改鞍座的结构形式,包括新增筋板、增加壁厚,使该设备的设计满足了相关规范的要求。结论通过修改鞍座的结构形式,确保其必要的安全功能得以实现,满足实际工程的需要。     

Prenatal diagnosis of Fukuyama congenital muscular dystrophy

Fukuyama congenital muscular dystrophy (FCMD) is characterized by infantile hypotonia, symmetrical generalized muscle weakness, and neuronal migration disturbances that result in changes consistent with cobblestone lissencephaly with cerebral and cerebellar cortical dysplasia. FCMD is recognized as an autosomal recessive genetic defect. Genetic counselling is recommended for parents at risk of having a child with FCMD. Given the high risk and overwhelming prospect of having another child with this incurable devastating condition leads many couples to consider prenatal diagnosis. In Japanese families, haplotype analysis using microsatellite markers is available. In non-Japanese families, DNA sequence analysis is available. Both disease-causing alleles of an affected family member must be identified before prenatal testing can be performed. Copyright © 2006 John Wiley & Sons, Ltd.     

江苏台网尾波持续时间震级的初步研究

利用江苏台网15个地震台的84个地震DD-1短周期记录资料,通过m_(Lg)震级与持续时间τ和震中距Δ相关,得出该区持续时间震级M_D公式:M_D=a_0+0.44(lgτ)~2+0.00036Δ+DM这里,a_0为与记录分向和尾波截止水平有关的常数;DM为台站校正值。M_D标度内部单台标准差为0.09～0.10震级单位,M_D相对m_(Lg)的单台标准差为0.16～0.17震级单位。     

面向突发事件智能建筑管理系统的集成结构

为了实现面向突发事件的智能建筑管理系统(IBMS),提出了智能建筑突发事件的抽象模型、基本运算操作及优先权分配机制,并根据突发事件监控和管理的目标,提出了IBMS的"分布式虚拟应急中心"集成结构.     

全新世青藏高原东部西南季风的演变

本研究报道一组新的西南季风代用指标,即泥炭中单一种属植物———木里苔草残体纤维素和泥炭混合植物残体纤维素的δ13C时间序列,所记录的青藏高原东部全新世气候变化。两记录表明,该区全新世的下限年龄约11200aBP(14C年龄约9900aBP);从约11200aBP起该区迅速进入湿暖的全新世阶段,季风活动迅速增强;在约10800～5500aBP期间,季风总体保持在强盛状态,但其间有4次突然减弱,气候变干冷;约从5500aBP起季风活动在波动中逐渐减弱,其中有4次减弱最明显。所有这8次气候的突然变化都与北大西洋浮冰事件一一对应。这种密切的相关关系表明,西南季风强度的波动可能是对全球变化,特别是对海洋热盐环流引起的地球南北方气候波动的所谓"跷跷板效应"的响应。     

基于事故序列图的大型油轮原油泄漏事故风险研究

美国海岸警卫队在利用IRA评估其装备总体风险的过程中,充分运用事故序列图帮助分析人员从整体层面认识事故、灾难从产生到发展的过程,查找事故中的各个要素,分析各要素在事故演变过程中的作用和影响。针对我国大型油轮原油泄漏事故风险日益增加的现状,介绍了事故序列图分析事故的原理,并结合典型事故案例,给出了防止大型油轮发生原油泄漏事故的措施,以及减轻事故影响的方法。     

移动式环保公厕车防震抗风安全性研究

移动式环保公厕车工作地点范围广、环境复杂,且没有地基固定,要求其具有特别高的抗地震能力、抗风载变形能力。利用有限元仿真的方法,研究了移动式环保公厕车在不同地震载荷、风力载荷条件下的变形和应力状况,分析了地震载荷和风力载荷对移动式环保公厕车破坏程度和破坏机理,对比了单一载荷作用与复合载荷作用的区别,从而为移动式环保公厕车使用环境的选择、安全检查的合理安排等提供有力的参考依据。