Successful DNA-based prenatal exclusion of juvenile neuronal ceroid lipofuscinosis

A family with two siblings, 10 and 8 years old, both with clinical and ultrastructural evidence of juvenile neuronal ceroid lipofuscinosis is described. The family was found to be informative for the restriction fragment length polymorphisms (RFLPs) detected by the probes pCJ52–95Ml (locus D16S148) and pCJ52-94Tl (locus D16S159) flanking the juvenile neuronal ceroid lipofuscinosis locus, CLN3. The parents were both heterozygous using these probes, while their two children with juvenile neuronal ceroid lipofuscinosis were both homozygous. Chorionic villi analysis showed that the fetus was heterozygous and had inherited the one allele of the mother which was not found in the two siblings. This suggested that the fetus had derived one healthy allele from the mother, the risk for a double crossing-over being less than 1 per cent. Electron microscopy showed no fingerprint inclusions in chorionic villi. The child was investigated at 6 months of age and found to be healthy, as new fingerprint inclusions were found at electron microscopy and no vacuolated lymphocytes were found in the blood smear. Due to the risk of heterogeneity, both DNA-based analysis and electron microscopy on chorionic villi are recommended for prenatal examination for juvenile neuronal ceroid lipofuscinosis.     

循环水水质自动控制系统

简要介绍循环水KPI型循环水水质自动控制系统的技术性能。     

characteristics of PM2.5 in rural areas of southern Jiangsu Province, China

Introduction Anumberofstudiesabroadhavedemonstratedthat atmosphericfineparticulatematter(PM2.5,dp<2.5μm)is notonlyassociatedwithatmosphericvisibilityreductionbut alsohasaclearcorrelationwiththenumberofdailydeathsandhospitalizationsasaconsequenceofpulmonarydisease(Chan,1997;Christoforous,2000;Schwartz,1996;Wilson,1997).On18July,1997,theU.S.EnvironmentalProtectionAgency(USEPA)promulgatednewandsevere standardsforfineparticulates(PM2.5)withthesizebelow2.5μm(15μg m3asannualaverage,65μg m…     

大同农村水环境污染原因分析及防治对策

文章在概述大同农村水环境状况的基础上,分析了其污染成因,并提出了保护与治理农村水环境的对策与建议.     

The curve of ion exchange ratio(%)-pH of the interaction between suspended particles with Cd(II) in the Yellow River

The curve of ion exchange ratio(%)-pH of the interactionbetween suspended particles with Cd(II) in the Yellow River wasstudied. The effects of lysine on this curve have been alsoinvestigated. The results showed that (1) Cadmium in Cd(OH)+ formin the suspended particles exchanges with the cations.The exchangeratio of Cd2+ is nearly at its greatest value in the range of pH(8.0-8.5) in natural aquatic system; (2) Ion exchange ratiodecreases as the concentration of Cd2+ raises from 8.9×10-6 mol/L to 2x8.9 x 10-6mol/L; (3) At the lysine concentration of 6.8x10-6 mol/L, it can promote the ion exchange ratio; (4) Adsorption of thesuspended particles to cadmium is weaker in seawater and Jin ShaRiver than in the Yellow River.     

十六位DAC转换器的研制

十六位DAC转换器是为MAT系列同位素质谱仪磁场控制关键部件DAC73K模块进行国产化而设计的,它能够与八位、或十六位微机相兼容,具有精度高、准确度好、温飘小等特点,它的研制成功不仅加速了MAT系列质谱仪国产化进程,而且在其它自动控制系统中得到广泛应用.     

安徽月山地区闪长岩类地质地球化学研究

安徽月山地区是长江中下游地区的重要成岩-成矿区段。本文通过岩石学、岩石化学和同位素地球化学等方面的研究，探讨和阐明了月山地区闪长岩类的形成环境、物质来源、源区特征、岩浆形成与演化机理等。     

厌氧-混凝工艺处理造纸厂终端废水试验研究

本文报告了用厌氧 -混凝工艺处理造纸厂终端废水的实验情况 ,在最佳实验条件下 ,废水从CODcr80 6 . 7m g/ L、BOD52 10 . 5 m g/ L、SS2 15 . 7m g/ L、OD4 650 . 35 0降至 CODcr6 1. 5 m g/ L、BOD52 7. 4m g/ L、SS17.3mg/ L、OD4 650 .0 11。实验结果表明对造纸厂终端废水的治理 ,本工艺是很有实用价值的一种方法     

GJB 150A淋雨试验强化试验程序数值模拟和试验装置设计研究

目的 研究GJB 150.8A—2009《军用装备实验室环境试验方法第8部分:淋雨试验》中强化试验程序的工程实现方法.方法 在标准解读的基础上,以强化试验程序用喷嘴为对象,建立数值模型,分析喷嘴在强化试验程序过程中的流场分布,并基于分析结果给出强化试验程序试验装置设计的思路和方法.结果 在喷嘴内,压力变化剧烈,极短距离内由276 kPa迅速降至101 kPa.水流经过喷嘴经历了激烈的加速过程,从距离喷嘴出口0.01～0.4 m,水流速度从251 m/s迅速降至17.8 m/s,到远场趋于稳定.结论 分析结果对指导强化试验程序的试验装置的设计和试验实施具有重要参考意义.     

城市垃圾填埋场渗滤液的水质特性

对垃圾填埋场渗滤液的主要有机污染物、水质影响因素及水质特性进行了较系统的分析