Fetal presentation of morquio disease type A

A fetus with mucopolysaccharidosis type IV A (Morquio type A) is described. The family had one affected child exhibiting symptoms of classical Morquio A disease, and late in the subsequent pregnancy prenatal diagnosis was requested. At 23 weeks' gestation, moderate ascites was detected by detailed ultrasound scan and keratan sulphate was found in the amniotic fluid. The pregnancy was terminated by prostaglandin induction and the diagnosis of mucopolysaccharidosis type IV A was confirmed by demonstration of a deficiency of N-acetylgalactosamine-6-sulphate (GalNac-6-S) sulphatase in cultured amniotic cells and in post-mortem fibroblast cultures. The activities of β-galactosidase and arylsulphatase A were normal, ruling out Morquio disease type B and multiple sulphatase deficiency. These results indicate that mucopolysaccharidosis IV A (a disease that predominantly affects the skeletal system) may produce ascites in the fetus to such an extent that it can be detected by ultrasound.     

磁处理水对红星苹果贮藏特性的影响

用磁处理水处理红星苹果能显著地保持其贮藏期的果实硬度和可溶性固形物的含量，对果实的呼吸强度有明显的抑制作用，从而可提高红星苹果的贮藏品质，延长果实寿命．说明这种处理是水果保鲜的一种有效途径．     

首钢特殊钢公司炼钢二厂电弧炉除尘设备技术改造

介绍了首钢特殊钢公司炼钢二厂7号电弧炉除尘设备改造成功的经验，总结并讨论了中小型电弧炉除尘系统存在的问题。     

切花保鲜原理与保鲜技术

本文综述了切花保鲜的主要原理及保鲜技术，内容包括：水分与切花衰老的关系及其保鲜技术，激素在切花衰老和保鲜作用中的最新研究进展，冷藏技术在切花贮藏保鲜中的应用，切花保鲜剂的种类及其组分和作用．     

油气储运设施事故风险指数模糊逻辑评估方法

油气储运设施风险是其事故发生概率和事故后果的综合度量,而事故概率和后果的定量评估结果往往是具有不确定性的数据,以确定性风险评估准则为基础的传统风险矩阵法和风险值法显然难以评估油气储运设施风险。为此提出开展油气储运设施事故风险的模糊逻辑推理法,首先,对风险矩阵的概率语言等级和损失语言等级的边界进行定量划分;然后,建立油气储运设施风险矩阵模糊集和模糊逻辑推理规则;最后,通过风险模糊推理运算和模糊风险解模糊化以确定油气储运设施的风险水平。实例应用与分析表明,利用推荐方法可得到较为详尽的风险数据信息,不但风险指数更加清晰,而且其所属风险等级类别也更加明确,评估结果能更好地指导油气储运设施的风险管理。     

原油储罐硫化氢脱除实验及应用

采用循环气提法脱除原油储罐中的硫化氢以达到降低储罐内硫化氢浓度的目的。某联合站卸油台零位罐的实验结果表明:经过7h的实验,零位罐观察口硫化氢浓度可由484mg/m3降低至9mg/m3。文章主要介绍了循环气提法在轮三联合站卸油台零位罐的实验过程、除硫剂的筛选及在某联合站原油储罐的应用情况。实践证明该方法能够有效降低储罐内硫化氢浓度。     

"有序"开采:北方地区地下水资源利用中两难选择的途径

北方干旱、半干旱地区地下水超采严重，水资源供需矛盾突出。为探索一条地下水合理开发利用的途径．本文提出了“有序”开采的思路：在来来几十年内．政府部门应根据人口，经济发展规划提出地下水资源需求量的控制计划．并给下属各地区下达取水配额。使储存资源的消耗处于有计划和监控范围内。有序开采应分阶段实现：第一阶段应以抑侧超采量的过快增长为目标；第二阶段应以需水总量的下降为目标；第三阶段应实现总需水量低于地下水系统补给资源量，使数十年超采造成的储存资源亏空逐年得以偿还。“有序”开采应注意三点：①超采必须限制在有限时间内；②开采活动应在政府的严格控制之下。任何超过计划配额的取水行为都是不允许的；③开采量和总需水量的下降，必须以节水为前提。发达国家的需水量变化经历证明。通过“有序”开采实现北方地区地下水可持续利用是可行的．     

A study on operation problems of hydropower plants integrated with irrigation schemes operated in Turkey

In this study, Hydroelectric Power Plants, which have been built and integrated with irrigation schemes by the State Hydraulic Works which is a government agency and private companies in Turkey, have been examined. Technical, environmental, structural and social problems encountered during their operation have been analyzed, and appropriate solution proposals have been presented in the study for a sustainable irrigation and Hydroelectric Power Plant operation. Consequently, Hydroelectric Power Plants which have been integrated with irrigation schemes should be operated efficiently and they should be operated in attenuation with the environment. However, when hydroelectric projects are developed without preparing their necessary integrated basin management plans, then this will cause environmental, operation and maintenance, administrative, monitoring and evaluation problems. In order to ensure sustainability of hydroelectricity production and also in order to use water resources more efficiently for irrigation; it is very important to find permanent solutions to these problems.     

邻避设施导致的资产损失评估研究——以南京市为例

随着居民环境和公众维权意识的提高,邻避冲突会逐步从感性的情绪表达过渡到理性的利益诉求。选择房地产作为度量邻避设施导致的资产损失的媒介,基于双重差分模型构建了邻避设施导致的资产损失评估方法。以南京市为例,选择55个有效住宅社区1980个样本作为处理组,选择25个商圈住宅社区900个样本作为对照组,研究了化工厂、污水处理厂、公墓和监狱四种典型邻避设施对周边资产价格的影响。研究发现:不同邻避设施对周边资产影响不同;化工厂、监狱、公墓和污水处理厂的影响程度依次为302、284、194和180;区域繁华因素等可以消除邻避设施的部分影响;邻避设施周边资产的市场表现仅满足"上涨时涨得少",而不满足"下跌时跌得多"的特点。