Thermosensitivity of red blood cells from Down's syndrome individuals

Biochemical disturbances of the reactive oxygen species metabolism revealed in subjects with Down's syndrome (DS), and the findings indicating that heat-induced cell alterations have been, at least, partly mediated by reactive oxygen species, made the elucidation of the response of trisomic cells to elevated temperatures of special interest. Kinetic analysis of cell-survival curves, accompanied by the flow cytometry and the scanning electron microscopy (SEM) examinations, and their relationship with the cell membrane fluidity, were undertaken. At each temperature (48-54 degrees C), Dq parameters, representing the ability to accumulate sublethal damages, were similar for both cell groups. D0 parameters (inverse leakage rates; D0 = 1/k) were greater for DS cells at each temperature below 54 degrees C. The haemolysis sensitivity ratio (HSR) showed that DS erythrocytes were, in average, 1.60 times more resistant to heat injury than those from normal subjects. Activation energies of haemolysis, calculated according to the Arrhenius equation, were similar both for normal (290.8 +/- 6.5 [kJ/mol]) and DS erythrocytes (288.0 +/- 5.5 [kJ/mol]). Flow cytometry studies showed that the scattering properties of intact DS erythrocytes (reflecting size, volume, shape and cell membrane surface morphology) were different than those of normal cells. Scanning electron micrographs and scattering diagrams obtained for cells submitted to heat stress (51 degrees C) confirmed that DS erythrocytes were more resistant, to a certain extent, to heat-induced disruption than normal cells. The steady-state fluorescence anisotropy of TMA-DPH (1-(4-trimethyl-ammoniumphenyl)-6-phenyl-1,3,5-hexatriene) showed that untreated DS erythrocytes had substantially lower fluidity (r = 0.356 +/- 0.008) of the outer monolayer of cell membranes as compared to normal cells (r = 0.324 +/- 0.011). The increase of the cell membrane fluidity during exposure to heat was observed. The greatest elevation of cell membrane fluidity occurred during the preleakage period, immediately upon the heat treatment and was considered as a rate-limiting step of heat-induced haemolysis.     

Jet impingement onto a tapered hole: Influence of jet velocity and hole wall velocities on heat transfer and skin friction

Jet impingement onto a hole with elevated wall temperature can be associated with the high‐temperature thermal drilling, where the gas jet is used for shielding the hole wall from the high‐temperature oxidation reactions as observed in the case of laser drilling. In laser processing, the molten flow from the hole wall occurs; and in the model study, the hole wall velocity resembling the molten flow should be accounted for. In the present study, gas jet impingement onto tapered hole with elevated temperature is considered and the heat transfer rates as well as skin friction at the hole wall surface are predicted. The velocity of molten flow from the hole wall determined from the previous study is adopted in the simulations and the effect of hole wall velocity on the heat transfer rates and skin friction is also examined. It is found that the Nusselt number and skin friction at the hole wall in the regions of hole inlet and exit attain high values. The influence of hole wall velocity on the Nusselt number and skin friction is found not to be very significant. Copyright © 2008 John Wiley & Sons, Ltd.     

气膜孔角度对动叶前缘冲击/气膜复合冷却的影响

本文针对GE-E3第一级动叶前缘的冲击/气膜复合冷却结构进行了热流耦合数值研究。采用标准k-ω湍流模型,分析了前缘气膜孔对称布置时,其角度对透平动叶前缘冲击/气膜复合冷却特性的影响;在五种冷气质量流量比(MFR=0.005,0.010,0.016,0.020,0.025)下,研究了气膜孔在不同角度(β=20°,25°,30°,40°,50°,60°)时的透平动叶前缘冷却换热效果。研究结果表明:在本文研究范围内,气膜孔角度越小,透平动叶前缘的平均综合冷却效率越高;随着冷气质量流量比增大,透平动叶前缘的平均综合冷却效率逐渐提高。     

涡轮叶片前缘阵列冲击冷却流动及传热特性数值研究

本文采用SST湍流模型模拟了类前缘通道内蒸汽射流阵列冲击冷却的流动与传热特性,分析了雷诺数(Re=10000~50000)、孔径比(d/H=0.5~0.9)和孔间距比(S/H=2~6)对流动及传热性能的影响规律,得到了相应的传热和摩擦关联式。结果表明:在不同雷诺数下,d/H从0.5到0.9变化时,通道压力损失系数降低了76%~79%,靶面平均努塞尔数降低了45%~49%;S/H从2增至6时,通道压力损失系数增加了1.64~1.92倍,靶面平均努塞尔数增加了54%~64%;增大d/H、减小S/H可有效提高类前缘通道蒸汽冲击冷却的综合热力系数。本文研究结果可为未来先进燃气轮机高温涡轮叶片蒸汽冷却结构的设计提供参考和借鉴。     

多化学组份反应气体流动的Godunov格式

本文将单介质气体流动的Goduoov方法推广到多化学组份气体流动的计算中,建立了多化学组份气体的间断分解公式以及任意四边形网格下的Godunov方法的差分格式,提出了处理自由边界的虚相法,应用第二类网格,计算了超音速射流及其冲击问题的几个算例,并且同实验结果进行了比较。     

真实火箭射流冲击流场中激波结构的实验研究

本文采用一种有效的激光-莫瑞光栅干涉系统首次拍摄到真实火箭燃气射流冲击流场的激波结构.解决了长期以来无法拍摄到高温、高速、强火焰光的火箭射流流场图像的问题。利用这一系统,研究了射流冲击流场中激波结构的一些变化规律,发现了一些新的激波结构。较系统地研究了欠膨胀射流冲击各种锥体的激波形态。     

Novel deletion of the E3A ubiquitin protein ligase gene detected by multiplex ligation-dependent probe amplification in a patient with Angelman syndrome

Angelman syndrome (AS) is a severe neurobehavioural disorder caused by failure of expression of the maternal copy of the imprinted domain located on 15q11-q13. There are different mechanisms leading to AS: maternal microdeletion, uniparental disomy, defects in a putative imprinting centre, mutations of the E3 ubiquitin protein ligase (UBE3A) gene. However, some of suspected cases of AS are still scored negative to all the latter mutations. Recently, it has been shown that a proportion of negative cases bear large deletions overlapping one or more exons of the UBE3A gene. These deletions are difficult to detect by conventional gene-scanning methods due to the masking effect by the non-deleted allele. In this study, we have used for the first time multiplex ligation-dependent probe amplification (MLPA) and comparative multiplex dosage analysis (CMDA) to search for large deletions affecting the UBE3A gene. Using this approach, we identified a novel causative deletion involving exon 8 in an affected sibling. Based on our results, we propose the use of MLPA as a fast, accurate and inexpensive test to detect large deletions in the UBE3A gene in a small but significant percentage of AS patients.     

A case of Budd-Chiari syndrome: Gd-EOB-DTPA-enhanced MR findings

Budd-Chiari syndrome (BCS) is a rare disorder caused by the obstruction of hepatic venous outflow, leading to sinusoidal congestion, ischemic injury to liver cells and portal hypertension. Long-term survival largely depends on whether hepatocellular carcinoma occurs. A recently available liver-specific contrast medium, gadolinium-ethoxybenzyl-diethylenetriamine penta-acetic acid (Gd-EOB-DTPA), reportedly has high diagnostic capability for detection of malignant liver tumors. However, there has been no report of the sue of Gd-EOB-DTPA-enhanced magnetic resonance imaging (MRI) for BCS. We present a case of chronic BCS who underwent both gadopentetate dimeglumine (Gd-DTPA) and Gd-EOB-DTPA-enhanced MRI. Hepatic congestion and edema were seen as slightly hypointense areas on Gd-EOB-DTPA-enhanced hepatobiliary-phase images, although these areas were observed as slightly hyperintense on previously obtained Gd-DTPA-enhanced delayed-phase image. Reduced uptake of Gd-EOB-DTPA by hepatocytes in the region of congestion or edema may account for this difference, which should be recognized in image interpretations.     

纳洛酮佐治婴儿捂热综合征38例分析

用纳洛酮佐治婴儿捂热综合征39例，总有效率达84．2％，两组随访年后后遗症发生率下降明显。