颅骨缺损早期修补对小儿的影响（附47例分析）

目的探讨颅骨缺损早期修补对小儿的影响．方法对47例小儿颅骨缺损使用钛网行颅骨修补手术的临床资料进行回顾性分析．结果47例小儿颅骨缺损早期行手术修补均获得了好的疗效．结论小儿颅骨缺损早期行手术修补有利于脑功能的康复，对头颅的发育影响较少，减少或避免了颅骨缺损相关综合征．参7．     

Molecular analysis of genomic DNA allows rapid,and accurate,prenatal diagnosis of peroxisomal D-bifunctional protein deficiency

Prenatal diagnosis was requested for a couple with a previous child affected by the peroxisomal disorder D-bifunctional protein deficiency. Prior analysis of the D-bifunctional protein cDNA sequence from the propositus had shown that it was missing 22 bp. This was subsequently attributed to a point mutation in the intron 5 donor site (IVS5+1G>C) of the D-bifunctional protein gene. Consistent with parental consanguinity, the patient was shown to be homozygous for this mutation, which is associated with loss of a Hph 1 restriction site in the genomic sequence. Prenatal testing of the fetus using genomic DNA isolated from uncultured amniocytes indicated that both alleles of the D-bifunctional protein had the IVS5+1G>C substitution. The peroxisomal defect was later confirmed biochemically using cultured amniocytes, which were found to have elevated levels of very long chain fatty acids (VLCFA). This is the first report of prenatal diagnosis of D-bifunctional protein deficiency using molecular analysis of genomic DNA. Copyright © 2002 John Wiley & Sons, Ltd.     

含缺陷在用压力管道的安全性分析

采用有限元分析软件-ANsYs软件,建立了7组不同情况下含平面缺陷的弯管有限元模型,对管道上裂缝进行了应力计算和分析,并采用GB／T19624—2004《在用含缺陷压力容器安全评定》标准对含裂缝缺陷的压力管道进行了简化评定。结果表明7组评定点均处于安全区域,故裂纹缺陷不影响管线正常运行。     

S-100 protein in amniotic fluid of anencephalic fetuses

S-100 protein, which is found essentially in the astrocytes of the nervous system, was assayed in amniotic fluids by Particle Counting Immuno Assay. It was present in 19 cases of anencephaly out of 26, in 1 case of open spina bifida out of 5 and in each of the 4 cases of fetal death, whereas it was not detected in the 48 control amniotic fluids collected between the 16th and the 35th week of gestation. Thirty-one amniotic fluids from fetuses with other congenital malformations were devoid of detectable S-100. The presence of S-100 in amniotic fluid of anencephalic fetuses can presumably be considered as a biological sign of necrosis of the exencephalic brain and seems specific to damage of the central nervous system accompanied by neural tube defect.     

Pallister Killian-mosaic tetrasomy 12 p syndrome. Another prenatally diagnosed case

A new case of mosaic tetrasomy 12 p (46, XY/47, XY, + i 12 p), diagnosed during pregnancy from ultrasonographic signs, is reported. We emphasize the peculiar position of the diaphragmatic hernia in this syndrome. Its presence or absence determines the vital prognosis and the age of diagnosis. The knowledge of its possible association with tetrasomy 12 p can contribute considerably to the neonatal diagnosis by directing the work of the cytogeneticist to tissue cultures which enable him to detect the presence of the tetrasomy.     

马钢四铁厂2600mm喷吹罐的检验和缺陷修复

介绍了 2 6 0 0mm喷吹罐的检验工艺过程 ,并对缺陷进行了修复     

Isoelectric focusing pattern of human amniotic fluid α-fetoprotein

Isoelectric focusing (IEF) of amniotic fluid α-fetoprotein (AFP) in thin-layer polyacrylamide gels containing 8 M urea followed by immunoblotting reveals at least nine bands, band I lying next to the cathode. Compared with 298 amniotic fluid samples from normal pregnancies, we found that the density of band V was increased in seven cases of fetal death. In 16 amniotic fluid samples from pregnancies with open neural tube defects (ONTO), band V disappeared or was markedly decreased. In seven cases with elevated AFP and positive acetylcholines-terase (AChE) due to contamination with fetal blood, no difference in pattern was observed compared with samples from normal pregnancies. It is suggested that IEF of AFP and subsequent immunoblotting are an apparently diagnostic test for ONTD and intrauterine fetal death (IUFD).