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| Name | Class |
|---|---|
| Shire | INDUSTRY |
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Using whole blood samples and plasma samples obtained from some hemophilia A patients with inhibitors, the investigators will perform the coagulation assessment in the co-presence of aPCC and factor VIII by comprehensive coagulation assays and flow chamber analysis under blood flow conditions.
Comprehensive coagulation assays using recently popular rotational thromboelastometry (ROTEM), factor Xa/thrombin/plasmin generation test, and coagulation wave analysis and recently developed flow chamber system under blood flow conditions (T-TAS®) will be used. A bypassing agent, aPCC (Feiba®) and recombinant factor VIII (Advate®) will be added ex vivo to whole blood and plasma samples from patients with hemophilia A with inhibitors to compare with the coagulation effects of aPCC in the presence of factor VIII and those of aPCC in its absence under the conditions close to the physiological condition. Furthermore, the difference in the coagulation effects by the difference in inhibitor epitopes will be examined.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Combination therapy | Effects of aPCC reaction in the presence of FVIII |
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| Measure | Description | Time Frame |
|---|---|---|
| Evaluation of coagulation effects using patients' plasmas by FXa/thrombin/plasmin generation tests and clot waveform analysis | up to 10 months | |
| Evaluation of coagulation effects using patients' whole bloods by ROTEM and T-TAS | up to 15 months |
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Inclusion Criteria:
Exclusion Criteria:
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We guess that there are approximately 200-300 hemophilia A patients with inhibitors in our country. We have approximately 20 out-patients in our hospital. Based on this information, we set up the patients subjects (n=10).
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Keiji Nogami, MD, PhD | Contact | +81-744-29-8881 | roc-noga@naramed-u.ac.jp |
| Name | Affiliation | Role |
|---|---|---|
| Midori Shima, MD, PhD | Nara Medical University | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Department of Pediatrics | Kashihara | Nara | 634-8522 | Japan |
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| ID | Term |
|---|---|
| D006467 | Hemophilia A |
| ID | Term |
|---|---|
| D025861 | Blood Coagulation Disorders, Inherited |
| D001778 | Blood Coagulation Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
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| D020147 | Coagulation Protein Disorders |
| D006474 | Hemorrhagic Disorders |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |