GLOBAL ASSAY OF COAGULATION & FIBRINOLYSIS IN RARE FACTOR DEFICIENCIES

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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.