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排序方式: 共有270条查询结果,搜索用时 125 毫秒
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Pseudo-Bernard-Soulier syndrome: thrombocytopenia caused by autoantibody to platelet glycoprotein Ib 总被引:1,自引:0,他引:1
The Bernard-Soulier syndrome is an inherited bleeding disorder that is due to a deficiency in platelet glycoprotein Ib. Bernard-Soulier platelets fail to agglutinate in response to ristocetin despite normal levels of factor VIII:von Willebrand factor. We report a patient who developed severe refractory thrombocytopenia postsurgically while receiving procainamide therapy. Thrombocytopenia was immune mediated since the patient's platelets bore high levels of antiplatelet antibody. Radioimmunoprecipitation studies demonstrated that the autoantibodies had specificity for platelet glycoproteins Ib and V as well as platelet HLA. The patient's plasma as well as purified immunoglobulin G completely inhibited the ristocetin-induced aggregation of normal platelets but did not inhibit adenosine diphosphate-induced aggregation. The laboratory studies revealed that this patient suffered from antibody-mediated thrombocytopenia with unusual characteristics that we have called pseudo-Bernard-Soulier syndrome. 相似文献
266.
Maria?DoulaptsiEmail authorView authors OrcID profile Emmanuel?Prokopakis Sven?Seys Benoit?Pugin Brecht?Steelant Peter?Hellings 《Clinical and translational allergy》2018,8(1):32
Background
A visual analogue scale (VAS) is a psychometric instrument widely used in the Rhinology field to subjectively quantify patient’s symptoms severity. In allergic rhinitis, VAS has been found to correlate well with the allergic rhinitis and its impact on asthma severity classification, as well as with rhinoconjunctivitis quality of life questionnaire. In chronic rhinosinusitis (CRS), total VAS score are often used to classify disease burden into mild, moderate, and severe, with few studies correlating VAS scores with more complex and validated instruments assessing disease-specific burden like Sino-Nasal Outcome Test (SNOT)-22.Methods
We correlated VAS scores for total and individual sino-nasal symptom with SNOT-22 scores in a randomly selected group of 180 CRS patients. Pearson’s rho was selected as a correlation coefficient for analysis.Results
VAS scores for total nasal symptom score and individual symptoms correlated significantly with SNOT-22, irrespective of VAS based subclasses for sino-nasal, ocular, and bronchial symptoms.Conclusions
VAS for total sino-nasal symptom severity might be used for assessing disease severity, monitoring the course of the disease, and can be used for treatment decisions and disease burden.267.
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Evdokimov NM Van Slambrouck S Heffeter P Tu L Le Calvé B Lamoral-Theys D Hooten CJ Uglinskii PY Rogelj S Kiss R Steelant WF Berger W Yang JJ Bologa CG Kornienko A Magedov IV 《Journal of medicinal chemistry》2011,54(7):2012-2021
After the initial discovery of antiproliferative and apoptosis-inducing properties of a camptothecin-inspired pentacycle based on a 1H-indeno[2',1':5,6]dihydropyrido[2,3-d]pyrimidine scaffold, a library of its analogues as well as their oxidized planar counterparts were prepared utilizing a practical multicomponent synthetic protocol. The synthesized compounds exhibited submicromolar to low micromolar antiproliferative potencies toward a panel of human cancer cell lines. Biochemical experiments are consistent with the dihydropyridine library members undergoing intracellular oxidation to the corresponding planar pyridines, which then inhibit topoisomerase II activity, leading to inhibition of proliferation and cell death. Because of facile synthetic preparation and promising antitopoisomerase activity, both the dihydropyridine and planar pyridine-based compounds represent a convenient starting point for anticancer drug discovery. 相似文献
269.
系统性红斑狼疮患者骨髓间充质干细胞的体外扩增及鉴定 总被引:1,自引:0,他引:1
目的:探讨系统性红斑狼疮患者骨髓间充质干细胞的体外培养方法、生物学功能、表面标记表达的异常与否。方法:选择2004—12/2005—12于南京医科大学第一附属医院风湿科初诊为系统性红斑狼疮患者5例,均为女性,年龄20~35岁,诊断符合美国风湿病学院(ARA)1997年修订的诊断标准,所有患者知情同意后,获取骨髓标本。采用密度梯度离心法和贴壁筛选法分离培养5例系统性红斑狼疮患者骨髓间充质干细胞,观察其形态学改变,测定其生长曲线,采用流式细胞仪鉴定骨髓间充质干细胞表面标记物及分析细胞周期。结果:5例系统性红斑狼疮患者骨髓间充质干细胞均分离培养成功,其中1例因出现细菌污染而终止培养。①传代细胞生长较原代要快,细胞在8~10d即可达到90%融合。对P1、P2代细胞生长曲线进行观察,发现这2代细胞具有以下共同特征:传代培养的潜伏期为1~3d,第4天起呈对数增长,至第7天达到高峰,之后进入平台期。②流式细胞术检测问充质干细胞细胞表面抗原显示,细胞高表达CD105、CD13、CD90、CD73,而CD34、CD45、CD38、CD33阴性,3代以后的骨髓间充质干细胞纯度达到了95%以上,基本上排除了骨髓单个核细胞和巨噬细胞的污染。③间充质干细胞的周期分析显示,扩增后的间充质干细胞9.46%的细胞处于S,G2/M期,90.54%处于G0/G1期。结论:系统性红斑狼疮患者骨髓间充质干细胞体外可有效分离培养,所培养扩增的细胞成分单一,且保持干细胞特性,适于以后临床应用。 相似文献
270.
目的:利用抑制性消减杂交技术,筛选出大鼠心肌缺血再灌注的差异表达基因,以期通过基因线索探讨其损伤机制。方法:实验于2006—03/10在中山大学中山医学院完成。①实验分组:Sprague-Dawley雄性大鼠40只,随机分为手术组,对照组,每组20只。②实验干预:手术组结扎左冠状动脉,心电图出现急性心肌梗死改变90min后,去除结扎线,再灌注60min,建立心肌缺血再灌注大鼠模型。对照组仅穿线不结扎,余同手术组。③取缺血区心肌,提取Total RNA,构建cDNA文库,利用抑制性消减杂交技术筛选差异表达基因,测序,登录Genbank寻找同源性基因。结果:共获得124个阳性结果,56个为高表达基因,68个为低表达基因,其中发现5个新的cDNA片段。其中能量代谢、物质运输、信号转导相关差异表达基因分别占所有差异表达基因的39.25%,15.89%,15.89%,并且主要变化为下调。结论:抑制性消减杂交技术是一种高效的筛选差异基因的方法。心肌缺血再灌注后基因变化涉及多种功能的基因,以能量代谢、物质运输、信号转导相关基因下调明显。 相似文献