EVALUATION OF MULTIPLE HEMOSTATIC PARAMETERS FOLLOWING DOSING OF RECOMBINANT

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目及 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 假设：在血友病患者中给予重组因子Vila 90 mcg/kg后，血小板收缩力（PCF）、凝块弹性模量（CEM）和凝血酶生成时间（TGT）正常化。这些参数将根据以下既定凝血参数进行变更：血栓弹力图（TEG）、旋转血栓弹力图（ROTEG）、体外凝血酶生成、凝血酶-抗凝血酶复合物、凝血酶原片段1.2、因子VII活性和D-二聚体。 具体目标： a.记录伴和不伴自发性凝血因子VIII抑制剂的血友病患者的基线TGT、PCF和CEM异常。其次，使用TEG 4记录凝血缺陷！> ROTEG4！凝血参数以及检测凝血酶生成、达特、凝血酶原片段1.2、因子7活性和D-二聚体的试验。 B.确定给予rFVIIa 90 mcg/kg后上述患者中TGT、PCF和CEM正常化的程度。其次，记录使用TEG 4！> ROTEG4！凝血参数，以及检测凝血酶生成、达特、凝血酶原片段1.2、因子7活性和D-二聚体的试验。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Hypothesis: Following dosing of recombinant Factor Vila 90 mcg/kg in hemophiliacs, platelet contractile force (PC F), clot elastic modulus (CEM) and thrombin generation time (TGT) normalize. These parameters will change comparably to the following established clotting parameters: Thromboelastography (TEG), Rotational Thromboelastography (ROTEG), in vitro thrombin generation, thrombin-antithrombin complex, Prothrombin Fragment 1.2, Factor VII activity and D-dimer. Specific Aims: a. Document baseline TGT, PCF and CEM abnormalities in hemophiliacs with and without spontaneous factor VIII inhibitors. Secondly, document clotting deficiencies utilizing the TEG4!> and ROTEG4!> clotting parameters as well as assays detecting thrombin generation, TAT, Prothrombin Fragment 1.2, Factor 7 activity and D-dimer. b. Establish the extent by which TGT, PCF and CEM normalize in the above patients following the administration of rFVlla 90 mcg/kg. Secondly, document the extent by which clotting deficiencies normalize utilizing the TEG4!> and ROTEG4!> clotting parameters, as well as assays detecting thrombin generation, TAT, Prothrombin Fragment 1.2, Factor 7 activity and D-dimer.