GLOBAL ASSAY OF COAGULATION & FIBRINOLYSIS IN RARE FACTOR DEFICIENCIES

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 随着对血栓形成和出血性疾病的认识日益分子化，可识别的血栓形成和止血疾病的数量也在扩大，但这些不同疾病的分子病因与其对凝血系统的净影响之间的理解差距仍在继续扩大。在有相似分子缺陷的患者中，临床表型往往有相当大的差异，导致患者护理方面的困难。血栓形成和止血领域的科学家和临床医生已经认识到，有必要对凝血系统进行全球评估，以帮助提取复杂或多个缺陷的影响，简化目前广泛而昂贵的凝血和纤溶诊断测试小组，并更好地为个别患者量身定制有关预防和治疗的管理指南和建议。我们假设，全局测定将对区分各种止血障碍的类型和严重程度敏感，S绘制的凝血和纤溶潜力曲线将有助于评估有和没有出血障碍病史的患者的出血风险。我们计划利用全球检测的结果来了解罕见止血障碍患者的临床表型。

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. As the understanding of thrombophilia and bleeding disorders have become increasingly molecular, the number of identifiable disorders of thrombosis and hemostasis has expanded, yet the gap in understanding between the molecular etiologies of these varied disorders and their net impact on the clotting system continues to widen. Among patients with similar molecular defects, there is often considerable variation in clinical phenotype, leading to difficulty regarding patient care. Scientists and clinicians in the field of thrombosis and hemostasis have recognized the need for a global assessment of the coagulation system to help distill the effects of complex or multiple defects, to streamline a presently extensive and expensive panel of diagnostic coagulation and fibrinolytic testing, and to better tailor management guidelines and recommendations regarding prophylaxis and treatment to individual patients. We hypothesize that the global assay will be sensitive to discriminate among various types and severities of hemostasis disorders and the assay s generated curves of coagulation and fibrinolytic potential will assist in the assessment of bleeding risk among patients both with and without histories of bleeding disorders. We plan to utilize the results of the global assay to understand the clinical phenotype of patients with rare disorders of hemostasis.