Haemophilia A: mutation type determines risk of inhibitor formation

The formation of factor VIII antibodies is a major problem for replacement therapy of haemophilia A patients. Antibodies occur in 5-30% of patients with severe haemophilia A. The reason for antibody formation is still unknown. In this study we correlate for the first time different factor VIII gene mutations, stop- and missense mutations, large and small deletions and intrachromosomal intron 22 recombinations to antibody formation. A total of 364 patients with known inhibitor status of our institute, of the database, and of 3 studies representing intron-22-inversion data are included. The results show that the risk for developing factor VIII antibodies is strongly related to stop mutations. large deletions and intrachromosomal recombinations. A probable explanation could be the complete lack of endogenous circulating factor VIII protein in these cases. Other factors that might be important for the pathogenesis of inhibitor formation, e. g. the antenatal period, as well as possible therapeutic effects, are discussed.     

旋波媒质基本参数的一种测试方法

本文提出了直接利用旋波材料板的Ｓ参数获得其基本参数的方法。通过自由空间测量旋波材料板的反射场和透射场，可以反演得到媒质的复介电常数、复磁导率、旋波量以及透射场的轴比和旋转角，测试程序简单，结果精度高。     

MR monitoring of focused ultrasonic surgery of renal cortex: experimental and simulation studies

The aim of the study was to test the hypothesis that magnetic resonance (MR) imaging-guided and -monitored noninvasive ultrasonic surgery can be performed in highly perfused tissues from outside the body. A simulation study was performed to evaluate the optimal sonication parameters. An MR-compatible positioning device was then used to manipulate a focused ultrasound transducer in an MR imager, which was used to sonicate kidneys of five rabbits at various power levels and different durations. Temperature elevation during sonication was monitored with a T1-weighted spoiled gradient-echo sequence. The simulation study demonstrated that a sharply focused transducer and relatively short sonication times (30 seconds or less) are necessary to prevent damage to the overlying skin and muscle tissue, which have a much lower blood perfusion rate than kidney. The experiments showed that the imaging sequence was sensitive enough to show temperature elevation during sonication, thereby indicating the location of the beam focus. Histologic evaluations showed that kidney necrosis could be consistently induced without damage to overlying skin and muscle. The study demonstrated that highly perfused tissues such as the renal cortex can be coagulated from outside the body with focused ultrasound and that MR imaging can be used to guide and monitor this surgery.     

我国研究生教育科研素质培养探究

科研素质是衡量研究生人才的标准之一,也是高级人才的必备素质之一.本文就研究生的科研素质要求进行研究,同时对我国研究生的科研素质的薄弱部分进行分析,提出相应的解决对策.     

脱硫灰渣的排放方式及利用

火电厂产生的脱硫灰渣的组成和性能与其排放方式有着密切关系。为使其得到较好的处置与利用 ,本文从排放方式方面进行探讨 ,并略述各种脱硫灰渣的性能与利用前景     

合成孔径雷达图像的恒虚警率目标检测

目的　对合成孔径雷达 (SAR)图像实施恒虚警率 (CFAR)目标检测 .方法　利用 K-Gamma和Weibull分布的杂波模型分别对海面和陆地的人造目标实施检测 ;同时 ,对不同杂波背景下 CFAR检测方法进行比较 .结果与结论　通过检测结果的比较 ,证实了 K-Gamma分布和 Weibull分布分别适合于海面和陆地杂波背景下的目标检测 ;在非均匀杂波背景中 ,有序统计量 (OS)较单元平均 (CA)方法具有优越性     

微计算机控制的造波系统

本文所述系统可在水池中产生单一频率规则波、周期不规则波及"随机波",用于海洋、造 船工程的模型试验,采用频域迭代法和成型滤波器法来产生模拟海浪.尽管这一控制系统是 专为产生模拟海浪而设计的,但其控制方案也可直接用于其他测试技术领域,以产生"随机" 信号.     

Highly purified omega-3-polyunsaturated fatty acids for topical treatment of psoriasis. Results of a double-blind, placebo-controlled multicentre study

We report the results of a multicentre, double-blind, placebo-controlled study of topical therapy with omega-3-polyunsaturated fatty acids (omega-3-PUFA) in 52 patients suffering from moderate plaque-type psoriasis. In each patient, two similar stable psoriatic plaques served as indicator lesions for the study. One indicator lesion was randomly assigned to treatment with topical preparations of highly purified omega-3-PUFA in one of two concentrations (1 or 10%), and the other was treated with placebo. Efficacy assessment was based on changes in local psoriasis severity index, area involved, erythema, desquamation, induration and pruritus. After 8 weeks of treatment, all indicator lesions had improved significantly, compared with baseline. However, no statistically or clinically relevant differences between the omega-3-PUFA-treated and the placebo-treated lesions were found. Therapy was well tolerated and, apart from one patient who developed perilesional eczema, no clinically relevant adverse events occurred. In conclusion, topical omega-3-PUFA were not effective in a randomized, placebo-controlled, double-blind setting. Results of non-blind trials should be (re-)considered with caution.     

Fluorescence in situ hybridization mapping of the cystic fibrosis transmembrane conductance regulator (CFTR) gene to 7q31.3

We have used the fluorescence in situ hybridization (FISH) technique to refine the localization of the cystic fibrosis transmembrane conductance regulator (CFTR) gene on human chromosome 7. The result shows that the gene is most likely located within band q31.3.     

The side chains responsible for the dimerization of the estradiol receptor by ionic bonds are lost in a 17 kDa fragment extending downstream from aa 303

Fragments of 32, 26 and 17 kDa of the porcine estradiol receptor were prepared, all of which contain the ligand-binding site. While dimers of the 32 and 26 kDa fragments like those of intact receptor can be dissociated by protonation, the dimer of the 17 kDa fragment obtained by trypsination of the 26 kDa fragment is resistant to lowering the pH from 7.0 to 6.5 and below. Its dissociation can be achieved by 0.5 M MgCl2 at pH 7.0. All fragments are recognized by the MAB 13H2 in Western blots. The antibody also reacts with native receptor and the three fragments, both in their monomer and dimer states. The combining ratios of antibody with receptor, or its fragments, in the monomer and dimer states and the weakening of the estradiol-receptor bond by antibody attachment support the back to back and head to toe model of receptor dimers.