氟伐他汀对慢性心力衰竭患者血浆假性血友病因子、D-二聚体及心功能的影响

目的:观察氟伐他汀对慢性心力衰竭(CHF)患者血浆假性血友病因子(v WF)、D-二聚体(D-D)及心功能的影响。方法:78例CHF患者随机均分为对照组与试验组。对照组患者给予强心苷类药、利尿药、血管紧张素转换酶抑制剂或血管紧张素受体拮抗药、β受体阻滞药等常规治疗;在此基础上,试验组患者给予氟伐他汀钠胶囊40 mg,晚餐后顿服。两组患者疗程均为28 d。观察两组患者治疗前后左心室射血分数(LVEF)、左心室舒张末期内径(LVEDD)、v WF、D-D水平及不良反应发生情况。结果:治疗后,两组患者LVEF水平均显著高于同组治疗前,且试验组高于对照组;LVEDD、v WF、D-D水平均显著低于同组治疗前,且试验组低于对照组,差异均有统计学意义(P<0.05)。两组患者治疗期间均未见明显不良反应发生。结论:在常规治疗的基础上,氟伐他汀可显著改善CHF患者的凝血功能,安全性较好。     

血栓闭塞性脉管炎患者血浆D-二聚体血管性假血友病因子的变化及临床意义

2002年5月至2007年3月我院采用后腹腔镜下肾脏部分切除术治疗直径≤4cm的肾肿瘤患者19例,现报告如下。 1资料与方法 1．11临床资料：本组19例,男性12例,女性7例,32～72岁,平均58．4岁。左侧11例,右侧8例。临床表现有症状者5例,其中3例轻微腰痛,2例肉眼血尿,其余14例均于体检发现。术前均行B超、泌尿系统CT平扫＋增强检查。肿瘤直径2．2～3．8cm,平均2．93cm,未见腹腔淋巴结、肾血管、腔静脉、门静脉受累的影像学证据,无肝、肺转移。     

自体骨髓干细胞移植治疗血栓闭塞性脉管炎效果观察

目的 观察自体骨髓干细胞移植治疗血栓闭塞性脉管炎的临床应用效果及安全性.方法 对2004年9月-2012年12月收治的9例血栓闭塞性脉管炎患者（9条患肢）于缺血肢体行自体骨髓干细胞肌内注射移植,移植治疗3个月后,采用视觉模拟评分（VAS）法评估患肢疼痛缓解情况,并观察间歇性跛行距离、踝肱指数（ABI）和足部溃疡愈合情况.结果 移植治疗3个月后疼痛缓解8例,9例VAS评分由术前（7.89＆#177;1.36）分降至（2.11＆#177;2.20）分,差异有统计学意义（P〈0.05）;与术前比较,间歇性跛行距离明显延长、ABI明显升高（P〈0.05）;2例足部溃疡1例痊愈,1例溃疡面积明显缩小.随访6～24个月,患者症状及ABI均无恶化,无不良反应发生.结论 自体骨髓干细胞移植治疗血栓闭塞性脉管炎安全性好,近、中期疗效确切.     

奥扎格雷治疗急性脑梗死患者的疗效及其对血浆D-二聚体和血管性假血友病因子的影响

目的：观察奥扎格雷治疗急性脑梗死（ACI）患者的近期疗效及其对血浆D-二聚体（D-D）和血管性假血友病因子（vWF）的影响。方法：42例ACI患者给予脱水、降压等常规综合治疗,在此基础上给予奥扎格雷氯化钠注射液80mg.100mL-1,静脉滴注,bid,连续2周。观察疗效,治疗前、后神经功能缺损程度评分（NDS）和日常生活活动能力评分（ADL）改善情况及不良反应;测定血浆D-D、vWF含量,并与30名健康体检者比较（对照组）。结果：治疗后,患者NDS和ADL评分均显著改善,与治疗前比较,差异有统计学意义（P〈0.05或P〈0.01）,总有效率为90.5%。治疗前,ACI患者血浆D-D、vWF含量均显著高于对照组（P〈0.01）,治疗后ACI患者血浆D-D、vWF含量显著降低（P〈0.01）,但仍与对照组有显著性差异（P〈0.05）。结论：奥扎格雷能显著降低ACI患者血浆D-D、vWF水平,显著改善ACI患者NDS和ADL,且耐受性较好。     

门诊糖尿病患者踝肱指数检测及危险因子分析

<正>下肢动脉病变(PAD)是糖尿病常见并发症,往往与全身大血管病变同步发生。踝肱指数(ABI)测定已广泛用于临床,可较早发现PAD[1]。笔者在糖尿病专科门诊开展了这项工作,并测定了几项可能的影响因素,现将结果报告如下。     

肝硬化患者血浆D-二聚体的变化与病情相关性

目的探讨血浆D．二聚体水平与肝硬化病重程度的关系。方法回顾性分析本科收洽的140例肝硬化患者临床资料,按Child—Pugh评分分级,分别测定患者血浆D-二聚体水平,分析其在不同分组间的差异。结果患者血浆D．二聚体水平越高,肝硬化病重程度越重,3组间比较均差异有统计学意义。结论血D-二聚体指标变化与肝硬化严重程度有相关性,它可以作为判断肝硬化预后的一个重要指标。     

血栓性疾病患者进行血浆D-二聚体检测的临床意义探讨

目的 探讨对血栓性疾病患者进行血浆D-二聚体检测的临床意义.方法 选择90例血栓性疾病患者作为观察组,同期50例体检健康者作为对照组.两组均进行血浆D-二聚体检测.对比两组血浆D-二聚体水平及阳性率;对比观察组中不同血栓性疾病患者的血浆D-二聚体水平及阳性率.结果 观察组患者血浆D-二聚体水平为(789.93±87.1...     

心房颤动患者血浆D-二聚体与纤维蛋白原变化

目的观察慢性心房颤动(房颤)患者血浆D-二聚体和纤维蛋白原水平的变化,并探讨其意义。方法测量48例房颤患者(其中风心病28例,冠心病20例)和30例正常人血浆D-二聚体(D-dimer)和纤维蛋白原(Fg)。结果房颤患者与正常人相比,血浆D-dimer浓度极显著升高(P<0.01),纤维蛋白原水平也显著升高(P<0.01)。风心病患者与冠心病患者之间D-dimer,Fg差异均无显著性意义(P>0.05)。结论房颤患者血浆D-dimer和Fg水平升高,可能与其高发血栓栓塞并发症有关。     

心肌梗死患者血浆D-二聚体水平检测

目的检测心肌梗死患者血浆中的D-二聚体含量,为患者的临床诊治及预后提供参考依据。方法选取心肌梗死患者30例进行临床观察,分别采取全自动血凝仪对患者急性期及恢复期的D-二聚体进行检测。结果患者在急性期的D-二聚体水平明显高于恢复期,存在显著性差异,有统计学意义(P<0.05)。结论心肌梗死患者血浆D-二聚体水平对于疾病的疗效观察及预后产生着至关重要的作用。     

血浆血管性血友病因子抗原、D-二聚体、纤维蛋白原/纤维蛋白降解产物检测在急性脑梗死中的诊断价值

目的 探讨血浆血管性血友病因子抗原(vWF∶Ag)、D-二聚体(D-D)、纤维蛋白原/纤维蛋白降解产物(FDP)检测在急性脑梗死中的诊断价值.方法 选取2019年4月至2020年12月上海仁济医院宝山分院收治的86例急性脑梗死患者作为观察组,选取2019年4月至2020年12月上海仁济医院宝山分院60例健康体检者为对照...     

von Willebrand factor, von Willebrand factor-cleaving protease, and shear stress

von Willebrand factor (VWF) is a multimeric plasma glycoprotein (GP) involved in platelet adhesion at the site of vascular damage, which acts as a bridge between the injured subendothelium and the platelet receptors. The multimeric structure of VWF allows it to support multiple interactions with platelets and endothelial components under high shear stress. Rapid flow conditions induce a conformational transition of the VWF molecule, thus allowing its functional binding domains to be exposed. A specific VWF-cleaving protease (ADAMTS-13) physiologically down regulates the multimeric size of newly released and circulating VWF in order to prevent unwanted platelet thrombus formation. The occurrence of microvascular platelet aggregation in thrombotic thrombocytopenic purpura, which is caused by an ADAMTS-13 deficiency, well-demonstrates the important role of the protease in regulating the adhesive activity of VWF. Better knowledge of VWF function would contribute to the development of novel anti-thrombotic strategies based on the selective inhibition of the VWF interaction with platelet receptors and endothelial components in areas of the circulation characterised by elevated fluid dynamic forces.     

Thrombospondin-1 in von Willebrand factor function

Thrombospondin-1 (TSP1), expressed in many cells and tissues is abundantly present in platelet alpha-granules, from where it is released upon platelet activation. Murine Tsp1(-/-) platelet studies have revealed that TSP1 is redundant for platelet aggregation, but that it reinforces platelet aggregate stabilization, especially in a shear field. von Willebrand factor (VWF), synthesized by megakaryocytes and endothelial cells is stored both in platelet alpha-granules and in endothelial Weibel-Palade bodies as ultralarge multimers. When released from endothelial cells, these multimers are temporarily retained on the endothelium, to be cleaved by the plasma protease ADAMTS13 into smaller and hemostatically less reactive multimers, released in plasma. This protease shows partial sequence identity with the type 1 (TSR1) and type 2 (TSR2) repeats of TSP1 and contains 1 TSR1 and 6 TSR2 repeats. TSP1, locally released by platelets, competes with ADAMTS13 during VWF proteolysis and controls the degree of VWF multimer processing. In addition, TSP1 and VWF both interact with the platelet GPIb/V/IX membrane complex, primarily in flow. These interactions control the recruitment of platelets to (sub) endothelial VWF and TSP1, exposed to the circulation, as a consequence of vascular inflammation and endothelial injury. TSP1-VWF interactions do not strictly enhance platelet recruitment and secreted TSP1 even weakly competes with the dynamic platelet rolling and adhesion onto VWF. Hence, TSP1 and VWF show partially related hemostatic functions, the most important one being the TSP1 role in the ADAMTS13 operated VWF multimer processing, in pro-inflammatory and thrombogenic conditions.     

急性脑梗死患者血小板聚集功能、血管性血友病因子、抗凝血酶及 D-二聚体测定的临床意义

目的：探讨急性脑梗死患者血小板聚集功能（ PAgT）、血管性血友病因子（ vWF）、抗凝血酶（ AT）和D-二聚体（ D-dimer）水平变化及临床意义。方法选用相应的方法和仪器测定112例脑梗死及80例健康对照者血（浆） PAgT、vWF、AT和D-dimer水平变化,同时对部分患者进行治疗前、后的对比分析。结果脑梗死患者血中PAgT、vWF、D-dimer等指标均明显高于健康对照组,AT活性较对照组显著降低,差异有统计学意义（P＜0．05或P＜0．01）。选取经治疗效果明显好转的78例脑梗死患者,出院前取空腹静脉血测定PAgT、vWF、AT、D-dimer等指标,并与治疗前对照,结果治疗后PAgT、vWF、D-dimer降低,AT活性升高,差异有统计学意义（P＜0．05或P＜0．01）。结论脑梗死患者体内存在明显的凝血及纤溶功能异常,与血管内皮损伤、血小板聚集功能增强、凝血及纤溶功能亢进、抗凝功能降低等多因素有关。 PAgT、vWF、AT、D-dimer可以作为脑梗死患者诊断、治疗监测和预后判断的参考指标。     

The safety of plasma-derived von Willebrand/factor VIII concentrates in the management of inherited von Willebrand disease

Until the mid-80s, cryoprecipitate has been the mainstay of treatment of patients with von Willebrand disease who were unresponsive to desmopressin. The advent of virally-inactivated factor VIII (FVIII) concentrates containing von Willebrand factor (VWF), originally devoted to hemophiliacs, provided a better therapeutic approach to von Willebrand disease. These VWF/FVIII concentrates were introduced in clinical practice after the positive results obtained in several prospective and retrospective clinical studies. They are safe and can be suitable also for home treatment. Allergic or anaphylactic reactions are limited to the rare patients with deletions of VWF gene. In repeated infusions during surgery, the dosage and timing of administration should be planned to keep FVIII below 150 – 200 U/dl to avoid any possible risk of thrombosis.     

Human plasma von Willebrand factor/factor VIII complex (Haemate P/Humate-P): in von Willebrand disease and haemophilia A

Haemate P/Humate-P is a pasteurised human plasma-derived concentrate containing coagulation factor VIII and a near-normal spectrum of von Willebrand factor multimers, including high-molecular weight multimers, for intravenous use in patients with von Willebrand disease or haemophilia A. Extensive clinical experience over the past 25 years has shown that Haemate P/Humate-P provides effective haemostatic control for the prevention and treatment of bleeds in patients with these conditions, with no confirmed cases of viral or prion transmission occurring during this time. In small prospective and retrospective noncomparative studies, Haemate P/Humate-P provided effective haemostatic control for the prevention and treatment of bleeding episodes in the vast majority of paediatric and adult patients with von Willebrand disease. Haemate P/Humate-P was generally well tolerated in patients with von Willebrand disease or haemophilia A.     

血管性血友病因子层析纯化方法研究进展

从血浆中分离纯化出各种有价值的蛋白是生物技术领域的重要产业。血管性血友病因子可从血浆中提取,能有针对性地治疗遗传性和获得性血管性血友病,为患者提供最佳的治疗手段。蛋白层析纯化法能得到纯度高,活性佳的蛋白分子,是血管性血友病因子提纯的主要方法。现综述离子交换法、亲和层析法和凝胶过滤法3种重要的纯化方法在血管性血友病因子制备中的应用。     

房颤患者血管性血友病因子水平及临床意义

目的 探讨心房颤动(房颤,AF)患者血液中血管性血友病因子(vWF)水平变化及其临床意义.方法 持续性AF患者39例,根据超声检查分为血栓阳性(22例)和血栓阴性(17例)两组,另选窦性心律组和止常对照组各20例.采用酶联免疫吸附双抗体夹心法(ELISA)测定血浆vWF水平.结果 vWF水平血栓阳性组明显高于其他组(P＜0.05),Logistic回归分析显示左房内径和vWF水平是AF患者血栓形成的独立危险因素(P＜0.05).结论 vWF水平是AF患者血栓形成的独立危险因素.     

Pharmacological characteristics of solid-phase von Willebrand factor in human platelets.

1. The pharmacological characteristics of solid-phase von Willebrand factor (svWF), a novel platelet agonist, were studied. 2. Washed platelet suspensions were obtained from human blood and the effects of svWF on platelets were measured using aggregometry, phase-contrast microscopy, flow cytometry and zymography. 3. Incubation of platelets with svWF (0.2 - 1.2 microg ml(-1)) resulted in their adhesion to the ligand, while co-incubations of svWF with subthreshold concentrations of ADP, collagen and thrombin resulted in aggregation. 4. 6B4 inhibitory anti-glycoprotein (GP)Ib antibodies abolished platelet adhesion stimulated by svWF, while aggregation was reduced in the presence of 6B4 and N-Acetyl-Pen-Arg-Gly-Asp-Cys, an antagonist of GPIIb/IIIa. 5. Platelet adhesion stimulated with svWF was associated with a concentration-dependent increase in expression of GPIb, but not of GPIIb/IIIa. 6. In contrast, collagen (0.5 - 10.0 microg ml(-1)) caused down-regulation of GPIb and up-regulation of GPIIb/IIIa in platelets. 7. Solid-phase vWF (1.2 microg ml(-1)) resulted in the release of MMP-2 from platelets. 8. Inhibition of MMP-2 with phenanthroline (10 microM), but not with aspirin or apyrase, inhibited platelet adhesion stimulated with svWF. 9. In contrast, human recombinant MMP-2 potentiated both the effects of svWF on adhesion and up-regulation of GPIb. 10. Platelet adhesion and aggregation stimulated with svWF were reduced by S-nitroso-n-acetyl-penicillamine, an NO donor, and prostacyclin. 11. Thus, stimulation of human platelets with svWF leads to adhesion and aggregation that are mediated via activation of GPIb and GPIIb/IIIa, respectively. 12. Mechanisms of activation of GPIb by svWF involve the release of MMP-2, and are regulated by NO and prostacyclin.     

Factor VIII, von Willebrand factor and platelet adhesiveness in diabetic retinopathy

Thirteen non-diabetic controls, 40 diabetics without retinopathy and 19 diabetics with retinopathy, approximately matched for age and sex, were studied for plasma levels of F VIII:C, vWF:Ag and R Cof, and platelet adhesiveness. F VIII:C was elevated in both diabetic groups compared with normal controls, but no differences were found between the diabetic groups. vWF:Ag was significantly higher in both diabetic groups than in normal controls, and it was also elevated in diabetics with retinopathy compared with those without. R Cof was higher in diabetics with retinopathy than in normal controls or in diabetics without retinopathy, but there were no differences between normal controls and diabetics without retinopathy. We could not find any differences in platelet adhesiveness between the groups. The results in the present study suggested that F VIII/vWF might play an important role in the pathogenesis of diabetic retinopathy.