A5008757028- Hemophilia Treatment and Research
- Blood Coagulation and Thrombosis Mechanisms
- Platelet Disorders and Treatments
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Chronic Myeloid Leukemia Treatments
- Hemostasis and retained surgical items
- Blood properties and coagulation
- Heparin-Induced Thrombocytopenia and Thrombosis
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Particle Accelerators and Free-Electron Lasers
- Cancer-related gene regulation
- Venous Thromboembolism Diagnosis and Management
- Blood groups and transfusion
- Advanced X-ray Imaging Techniques
- Gyrotron and Vacuum Electronics Research
- Monoclonal and Polyclonal Antibodies Research
- Antiplatelet Therapy and Cardiovascular Diseases
- Particle accelerators and beam dynamics
- Acute Lymphoblastic Leukemia research
- Protease and Inhibitor Mechanisms
- Crystallography and Radiation Phenomena
- Structural Engineering and Vibration Analysis
- CAR-T cell therapy research
- Acute Myeloid Leukemia Research
- Kawasaki Disease and Coronary Complications
Nara Medical University
2016-2025
Nara Medical University Hospital
2013-2024
Pediatrics and Genetics
2022
Health Sciences University of Hokkaido
2020
Nihon University
2010-2019
Tokyo Institute of Technology
2000-2015
Tokyo Metropolitan University
2003-2013
Fundación Juan March
2010
May Institute
2010
Kaketsuken (Japan)
2008
In patients with severe hemophilia A, standard treatment is regular prophylactic and episodic intravenous infusions of factor VIII. However, these treatments are burdensome, especially for children, may lead to the formation anti–factor VIII alloantibodies (factor inhibitors). Emicizumab (ACE910), a humanized bispecific antibody mimicking cofactor function VIII, was developed abate problems.
Efanesoctocog alfa provides high sustained factor VIII activity by overcoming the von Willebrand factor-imposed half-life ceiling. The efficacy, safety, and pharmacokinetics of efanesoctocog for prophylaxis treatment bleeding episodes in previously treated patients with severe hemophilia A are unclear.We conducted a phase 3 study involving 12 years age or older A. In group A, received once-weekly (50 IU per kilogram body weight) 52 weeks. B, on-demand 26 weeks, followed primary end point was...
Emicizumab is a bispecific antibody that mimics the cofactor function of activated factor (F) VIII. It prevents bleeds in patients with congenital hemophilia A regardless inhibitor status; however, no prospective clinical studies have been conducted for emicizumab acquired (PwAHA).To describe primary analysis results from prospective, multicenter, open-label phase III study evaluating efficacy, safety, and pharmacokinetics PwAHA (AGEHA; JapicCTI-205151).Emicizumab was administered...
Concizumab is an anti-tissue factor pathway inhibitor monoclonal antibody designed to achieve hemostasis in all hemophilia types, with subcutaneous administration. A previous trial of concizumab (explorer4) established proof concept patients or B inhibitors.We conducted the explorer7 assess safety and efficacy inhibitors. Patients were randomly assigned a 1:2 ratio receive no prophylaxis for at least 24 weeks (group 1) 32 2) nonrandomly (groups 3 4). After treatment pause due nonfatal...
Emicizumab, a humanised bispecific antibody recognising factors (F) IX/IXa and X/Xa, can accelerate FIXa-catalysed FX activation by bridging FIXa in manner similar to FVIIIa. However, details of the emicizumab-antigen interactions have not been reported so far. In this study, we first showed surface plasmon resonance analysis that emicizumab bound FIX, FIXa, FX, FXa with moderate affinities (KD = 1.58, 1.52, 1.85, 0.978 µM, respectively). We next immunoblotting recognised antigens' epidermal...
Abstract Introduction Emicizumab is a recombinant humanized bispecific monoclonal antibody mimicking the cofactor function of activated factor VIII. Aim In this multicentre, open‐label study (HOHOEMI), we evaluated efficacy, safety and pharmacokinetics emicizumab in Japanese paediatric patients aged <12 years with severe haemophilia A without VIII (FVIII) inhibitors. Methods was administered subcutaneously, four loading doses 3 mg/kg every week followed by maintenance 2 weeks (Q2W) or 6 4...
Emicizumab (ACE910), a recombinant humanized bispecific monoclonal antibody, provides factor VIII (FVIII) cofactor bridging function to restore hemostasis in people with hemophilia A. In phase 1 trial involving 18 Japanese patients severe A, once-weekly subcutaneous administration of emicizumab 0.3, 1, or 3 mg/kg (cohorts 2, and 3, respectively) was well tolerated substantially reduced annualized bleeding rates (ABRs) the presence absence FVIII inhibitors. The current study represents an...
Abstract Introduction The efficacy and safety of recombinant factor VIII Fc fusion protein (rFVIIIFc) as an extended half‐life treatment for severe haemophilia A were demonstrated in the Phase 3 A‐LONG Kids studies. Eligible subjects who completed could enrol ASPIRE (NCT01454739), open‐label extension study. Aim To report long‐term rFVIIIFc with enrolled ASPIRE. Methods Previously treated received one or more following regimens: individualized prophylaxis (IP), weekly prophylaxis, modified...
summary Normal haemostasis is maintained by a controlled balance between coagulation and fibrinolysis, involving thrombin plasmin the respective key enzymes. Simultaneous evaluation of both enzymes facilitates, therefore, an overall understanding normal pathological haemostasis. Combined generation (T/P-G) assays have been recently described, we adapted technique to investigate interplay fibrinolysis in patients with various haemostatic disorders. Our modified T/P-G was initiated addition...
We previously identified a factor (F)VIII molecular defect associated with an R2159C mutation in the C1 domain (named "FVIII-Ise") together undetectable FVIII antigen (FVIII:Ag) levels measured by two-site sandwich ELISA using anti-C2 alloantibody (alloAb). The patient had clinically mild hemophilia A, and his reduced FVIII:C correlated FVIII:Ag monoclonal antibodies (mAbs) A2 A2/B epitopes, suggesting that modified C2 antigenicity. To investigate functional structural characteristics of...