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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.  相似文献   
996.
The design and evaluation of an optically triggered, fully integrated sample and hold circuit (OS/H) is described. Measured results are presented that demonstrate operation of this circuit at 250 Ms/s and with effective resolution approaching 8 bits. The integrated circuit, which measures 2.1 mm×1.4 mm, is realized in -1.0-V threshold, 20-GHz ft GaAs MESFET technology, consumes approximately 200 mW of power, and requires one optical address. The OS/H will find applications in high precision, hybrid, and integrated signal processing systems where high speed, high levels of parallelism, and low timing jitter are important. Measured results of a series photoconducting (Auston switch) OS/H realized in the same technology are presented for comparison purposes  相似文献   
997.
Recent developments of stability control in mines, essentially based on Ge-doped fiber Bragg gratings (FBG) are reported including results about the different aspects of the system: accurate characterizations of FBG, sensor network topology and multiplexing method, user interface design and sensor packaging  相似文献   
998.
This ecologic study aimed to investigate whether differences in population mortality from lung, stomach and colorectal cancer among the 16 cohorts of the Seven Countries Study could be explained by differences in the average intake of anti-oxidant (pro)vitamins. In the 1960s, detailed dietary information was collected in small sub-samples of the cohorts by the dietary record method. In 1987, food-equivalent composites representing the average food intake of each cohort at baseline were collected locally and analyzed in a central laboratory. The vital status of all participants was verified after 25 years of follow-up. The average intake of vitamin C was strongly inversely related to the 25-year stomach-cancer mortality (r = -0.66, p = 0.01), also after adjustment for smoking and intake of salt or nitrate. The average intake of alpha-carotene, beta-carotene, and alpha-tocopherol were not independently related to mortality from lung, stomach or colorectal cancer, nor was vitamin C related to lung and colorectal cancer.  相似文献   
999.
Mineralization occurred both in fetal rat calvarial cells and UMR 106 osteoblastic cells when they were cultured in medium containing L-ascorbate and beta-glycerophosphate as evidenced by von Kóssa staining as well as deposition of calcium ions and inorganic phosphate in the cells. When compared with corresponding non-mineralized cell cultures, both the mineralized cultures of calvarial cells and UMR 106 cells did not exhibit any change in intracellular bone-specific alkaline phosphatase activities which were measured by wheatgerm lectin precipitation method. Our results support the hypothesis that mineralization may not exert any direct negative feedback on matrix protein synthesis in osteoblasts during bone formation.  相似文献   
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