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The diagnosis of albinism is indicated by the presence of visual pathway misrouting in which temporal retinal fibers erroneously decussate at the optic chiasm disrupting the normal topographical distribution of retinal geniculate-cortical projections. Detection of misrouted fibers is effected by non-invasive electrophysiological assessment of the topographical representation of the visual evoked potential (VEP) following full field monocular stimulation. By combining appropriate state defined neonatal recording procedures with the albino VEP test paradigm, the presence of aberrant optic pathway projections was detected in a five-day-old full-term infant. The electrophysiological signature pathognomonic to albinism was observed within a long (300 ms) latency window of an otherwise normal neonatal luminance flash response. The results of this study indicate that the VEP misrouting test can be extended to reliable albino diagnosis within the neonatal period. 相似文献
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C E Hack J Wagstaff R J Strack van Schijndel A J Eerenberg H M Pinedo L G Thijs J H Nuijens 《Thrombosis and haemostasis》1991,65(5):497-503
Patients treated with high doses of interleukin-2 (IL-2) because of cancer, develop hemodynamic and vasopermeability changes, that resemble those observed in sepsis. These patients thus provide a unique opportunity to study the early events in the development of septic shock. We analysed the changes that occurred in the contact system of coagulation in plasma from 4 patients, who together received seven 12-day cycles of high doses of IL-2. Levels of factor XII and prekallikrein during the cycles progressively fell to 50 and 30% of their initial levels, respectively, whereas significant increases in plasma factor XIIa- and kallikrein-C1-inhibitor complexes were not observed (in 3 out of 211 samples slightly increased levels of both complexes were found). The reductions in factor XII and prekallikrein were only in part due to protein leakage, since levels were still significantly lower, i.e., 80 and 50%, respectively, when corrected for albumin decreases. Levels of high molecular weight kininogen (HMWK) also decreased during IL-2 therapy, however, this decrease paralleled that of albumin. SDS-PAGE analysis of plasma HMWK did not reveal increased cleavage of this protein. The reduction of factor XII and prekallikrein, corrected for protein leakage, significantly correlated with albumin levels and inversely with daily cumulative weight gain in the patients. Thus, we demonstrate that factor XII and prekallikrein decrease during IL-2 therapy. As these decreases, already observed after 1 day treatment, were disproportional to that of albumin, a negative acute phase reactant, and correlated with signs of the vascular leak syndrome, we favor the explanation that they reflected activation rather than a decreased synthesis of the contact system proteins.(ABSTRACT TRUNCATED AT 250 WORDS) 相似文献
35.
Alcoholic liver disease: an IgA-associated disorder 总被引:1,自引:0,他引:1
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Mo Weijtens Anke van Spronsen Anton Hagenbeek Eric Braakman Anton Martens 《Human gene therapy》2002,13(2):187-198
Graft-versus-host disease (GvHD), a major complication of allogeneic bone marrow transplantation, has been ascribed to mature T cells in the graft. Because T cells play an important role in engraftment of the bone marrow and decrease the probability of relapse of leukemia, a treatment strategy was developed to preserve the benefits of T cells in the graft and to control the severe complications of GvHD. This can be accomplished by the genetic modification of donor T cells with a suicide gene that allows their selective in vivo elimination and subsequently the abrogation of GvHD. For clinical benefit the alloreactivity of herpes simplex virus thymidine kinase (HSV-TK) gene-transduced T cells should be retained. Therefore, we investigated the influence of gene transduction and the selection procedure on T cells. We demonstrated that activation and culturing of T cells reduce their capacity to induce lethal GvHD in an allogeneic rat bone marrow transplantation model. Furthermore, positive immunomagnetic selection of gene-transduced T cells resulted in loss of the GvHD-inducing capacity of HSV-TK(+) T cells directly after MACS (magnetic cell sorting) selection; this loss could be recovered by a 1-day expansion of the selected T cells. No effect on alloreactivity was observed to be caused by the gene transduction procedure. Our study resulted in the development of an optimized culture and gene transduction protocol with preservation of T cell alloreactivity. Treatment of transplanted rats with ganciclovir resulted in a rapid reduction in the number of HSV-TK(+) T cells in the peripheral blood and in increased survival of the animals. 相似文献
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目的:优选散结消肿贴中大黄、重楼的渗漉提取工艺。方法:以总固体物得率和总蒽醌得率为指标,用正交实验优选。结果:工艺中影响最大的因素是乙醇浓度,其次是乙醇用量,浸泡时间影响较小。最佳工艺条件为乙醇浓度65%,乙醇用量8倍量。浸泡时间24h。结论:渗漉法操作简单,成份破坏少,用优选所得条件进行提取,总蒽醌和总固体物得率均较高,优选结果可用于散结消肿贴中大黄、重楼的提取。 相似文献
39.
膳食黑米皮抑制高脂诱导的家兔动脉粥样硬化形成及其机制的初步探讨 总被引:1,自引:0,他引:1
目的 探讨膳食黑米皮对高脂诱导的家兔动脉粥样硬化形成的影响及其作用机制。方法 24只雄性新西兰大耳白家兔,按体重随机分为3组,分别饲以正常基础饲料、高脂饲料(含96%基础饲料、0.5%胆固醇、3.5%猪油)、黑米皮饲料(含 91%基础饲料、0.5%胆固醇、3.5%猪油、5%黑米皮),实验期为 60d。用图像分析法测定各组家兔主动脉脂质斑块面积,并分析家兔氧化与抗氧化状态。结果 黑米皮实验家兔的主动脉壁斑块面积较高脂组低66%(P<0.001);黑米皮组实验家兔的主动脉壁8-ohdG含量和血清及主动脉壁中MDA水平也低于高脂组(P<0.05 );而红细胞及主动脉壁中SOD水平和血清α-生育酚含量在两个处理组之间差异无显著性。结论 膳食黑米皮可以抑制高脂诱导的家兔动脉粥样硬化形成,其机制可能与之降低机体氧化应激水平及过氧化脂质生成有关。 相似文献