关联维数在机械设备故障诊断中的应用研究

研究了混沌与分形的特征参数———关联维数的计算方法和参数的选择。以滚动轴承在正常、外圈故障、滚动体故障、内圈故障4种状态下的信号特征为标准样本,以其运行的实测信号为例,对时域信号进行了频谱分析,并进一步进行了关联维数分析。通过对滚动轴承振动信号的关联维数分析,证明了该轴承在4种不同标准状态下具有明显不同的关联维数特征。因此,按照相关性的大小,就可诊断出实测信号属于外圈故障状态信号。研究结果表明,关联维数分析方法在设备状态监测与故障诊断中,尤其是在非线性系统的故障诊断中显示出其独特的优势,具有较为广阔的应用前景。     

Activity and multiple forms of α-L-fucosidase and hexosaminidase in chorion biopsy specimens and some fetal organs

It was shown that activities and isoenzyme patterns of x-L-fucosidase and hexosaminidase were similar in biopsy specimens of chorion obtained immediately before induced abortion in normal pregnancy and in chorion tissue itself. A comparative study of isoenzyme patterns of these glycosidases showed their similarity in chorion and human fetal kidney, liver and lungs. The data obtained may be applied to the investigation of the multiple forms of α-L-fucosidase and hexosaminidase in chorion biopsy specimens for the prenatal diagnosis of fucosidosis (α-L-fucosidase deficiency). Tay-Sachs diseases (hexosaminidase A deficiency) and Sandhoff disease (deficiency of hexosaminidase A and B).     

Prenatal diagnosis of Niemann–Pick diseases types A,B and C

Prenatal diagnosis of Niemann–Pick disease types A and B is routinely accomplished by sphingomyelinase assay. For Niemann–Pick type C disease, demonstration of an abnormal intracellular cholesterol trafficking is a complex procedure, and mutational analysis (NPC1 or NPC2/HE1 gene), whenever feasible, represents a major advance. Copyright © 2002 John Wiley & Sons, Ltd.     

Detection and false-positive rates of maternal serum markers for Down syndrome screening according to maternal age in women over 35 years of age. A study of the agreement of eight dedicated software packages

Maternal serum markers for trisomy 21 screening (MSS) can be assayed in women ≥35 years in an attempt to reduce the need for invasive procedures and thereby avoid their side effects. Our objective was to compare, in women ≥35, eight different software packages dedicated to second trimester MSS, thus providing reliable data for patient counselling. A simulation study was carried out on 189 sera from women with Down syndrome fetuses and 11 962 sera from mothers of unaffected babies. The first step was to estimate the joint distribution of alpha-fetoprotein (AFP) and free beta-human chorionic gonadotrophin (β-hCG). The second step was to calculate trisomy 21 detection and false-positive rates for each software according to maternal age (35–45 years), using the usual 1:250 risk threshold. Agreement between software packages was measured using 2×2 kappa coefficients. Detection rates and false-positive rates increased with maternal age. Depending on the software, 57–71% detection rates were achieved at 35 years with 12–18% false-positive rates. At 45 years, 61–100% detection rates were achieved with 66–95% false-positive rates. Up to 39 years, all softwares were concordant (kappa coefficients >0.75). In the range 35–45 years, false-positive and detection rates increased substantially with maternal age and differences between software packages are observed. Copyright © 2002 John Wiley & Sons, Ltd.     

Ultrasound diagnosis of structural abnormalities in the first trimester

The advances in ultrasound technology have made it possible to identify fetal structural abnormalities and genetic syndromes in the first trimester. First trimester prenatal diagnosis of fetal central nervous system, renal, gastrointestinal, cardiac, and skeletal abnormalities is reviewed. Copyright © 2002 John Wiley & Sons, Ltd.     

Prenatal diagnosis of the Hunter syndrome and the introduction of a new fluorimetric enzyme assay

Prenatal diagnosis of the Hunter syndrome (mucopolysaccharidosis type II; MPS II) is preferably achieved by the assay of iduronate-2-sulphate sulphatase (IDS) in uncultured chorionic villi (CV) as this allows early (12th week), rapid (2–3 days) and reliable results. We summarize the results of 174 prenatal analyses in the past 30 years, using various methods such as radiolabelled sulphate incorporation in amniotic fluid (AF) cells, glycosaminoglycan (GAG)-electrophoresis in AF and IDS assay in CV, CV-cells, AF and AF-cells. Twenty-seven fetuses with MPS II were diagnosed after finding clearly abnormal results in pregnancies with a male fetus; very low IDS activity has also been measured in some pregnancies with a (heterozygous) female fetus, emphasizing the need to combine enzyme assay with fetal sex determination. IDS activity has until recently been assessed by a cumbersome radioactive enzyme assay. Here we describe the use of a novel fluorigenic 4-methylumbelliferyl substrate, which allows a sensitive, rapid and convenient assay of IDS activity and reliable early prenatal diagnosis. This novel IDS assay was validated in retrospective analyses of 14 CV, CV-cell, AF and AF-cell samples from affected pregnancies in addition to prospective prenatal diagnosis in eight pregnancies at risk with one MPS II-affected fetus. Copyright © 2002 John Wiley & Sons, Ltd.