Hemorrhagic, Coagulopathic, and Thrombotic (HECTOR) Complications Among Critically-Ill Patients with COVID-19: An International COVID-19 Critical Care Consortium Study
Background: Hemorrhage, coagulopathy and thrombosis (HECTOR) are reported complications of coronavirus disease 2019 (COVID-19); however, more information is needed on the prevalence of these complications…Sirtuin 1 as an endogenous inhibitor of NETosis
Background: Neutrophil extracellular traps (NETs) are implicated in thrombosis, stroke, and delayed wound healing, conditions that are prevalent in diabetes. While diabetes is known to…Intermediate versus low-dose low-molecular-weight heparin in pregnant and postpartum women with a history of venous thromboembolism (Highlow Study)
Background: Pregnancy-related venous thromboembolism (VTE) is a leading cause of maternal morbidity and mortality. Thromboprophylaxis is indicated in pregnant women with a history of VTE. The…Platelets as Versatile Effectors of Thromboinflammation in Chronic Myeloproliferative Neoplasms
Background: Patients with Philadelphia (Ph)-negative myeloproliferative neoplasms (MPN), such as polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF), often elicit unique clinical features, such…Roles of FVIII in endothelial cell biology more than a coagulation factor
Background: Hemophilia A (HA) is a rare bleeding disorder caused by the absence or dysfunction of Factor FVIII (FVIII). Clinical manifestations are spontaneous bleedings that…Relationship between transgene-produced FVIII and bleeding rates 2 years after gene transfer with valoctocogene roxaparvovec: Results from GENEr8-1
Background: Valoctocogene roxaparvovec (AAV5-hFVIII-SQ) gene transfer reduced bleeding through endogenous factor VIII (FVIII) production over 2 years in people with severe hemophilia A. The relationship…Efficacy, Safety, and Pharmacokinetics of Once-Weekly Efanesoctocog Alfa (BIVV001) Prophylaxis in Previously Treated Patients With Severe Hemophilia A: Results From the Phase 3 XTEND-1 Study
Background: Efanesoctocog alfa is a new class of factor VIII (FVIII) replacement designed to overcome the von Willebrand factor-imposed half-life ceiling. Aims: To evaluate the…Concizumab Prophylaxis in Patients With Haemophilia A or B With Inhibitors: Efficacy and Safety Results From the Primary Analysis of the Phase 3 explorer7 Trial
Background: Concizumab is a subcutaneously administered anti-tissue factor pathway inhibitor (TFPI) antibody in development as once-daily prophylaxis for all haemophilia patients. explorer7 (NCT04083781) primary analysis…Antiplatelet effects of inhibiting coagulation factor XI in a non-human primate model of atherosclerosis
Background: Inhibition of coagulation factor (F)XI represents a potentially safe and effective strategy to treat thromboinflammatory conditions in which platelets play a prominent role, including…A Phase 3 study (ATLAS-PPX) to evaluate efficacy and safety of fitusiran, an siRNA therapeutic, in people with haemophilia A or B who have switched from prior factor or bypassing agent prophylaxis
Background: Fitusiran, a subcutaneous (SC) investigational siRNA therapeutic, targets antithrombin to rebalance haemostasis in people with haemophilia A or B (PwHA/B), irrespective of inhibitor status.…Improvements in Health-Related Quality of Life in Adults with Severe or Moderately Severe Hemophilia B After Receiving Etranacogene Dezaparvovec Gene Therapy
Background: Despite current prophylactic therapy for hemophilia B, breakthrough bleeding still occurs negatively affecting health-related quality of life (HRQoL). The pivotal Phase 3 HOPE-B trial…In vivo murine studies demonstrate that neutrophil activation by anti-NAP2 antibodies contributes to vaccine-induced immune thrombocytopenia and thrombosis (VITT)
Background: VITT involves thrombocytopenia and thrombosis post-initial anti-SARS-CoV-2 adenoviral vaccination. Most patients are found to have platelet-activating antibodies to the chemokine, platelet factor 4 (PF4)…Base and Prime editing of DNA as a new therapeutic option for Hemophilia A
Background: Replacement therapy as well as non-replacement therapy, albeit significantly improved Hemophilia A (HA) treatment, do not provide a definitive cure and still have limitations.…Cooperation between PF4-specific antibodies in heparin-induced thrombocytopenia: a new mechanism potentially contributing to hypercoagulability and thrombosis
Background: Heparin-induced thrombocytopenia (HIT) is a severe complication of heparin therapy frequently associated with thrombosis. IgG antibodies against heparin-modified platelet factor 4 (anti-PF4/H) play a…The Prekallikrein and Factor XII Heavy Chains in Surface-Independent Reactions.
Background: The plasma zymogens prekallikrein and factor XII (FXII) are reciprocally converted to their protease forms, kallikrein and FXIIa, when blood is exposed to surfaces…Persistence of Ad26.COV2.S-associated VITT and specific detection of VITT antibodies
Background: Some COVID-19 vaccinated individuals develop anti-platelet factor 4 (PF4) antibodies that cause thrombocytopenia and thrombosis; a rare syndrome referred to as vaccine-induced immune thrombotic…Elucidating the Mechanism Behind the AAV-Derived Factor VIII Assay Discrepancy
The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
Background: Hemophilia A (HA) AAV-mediated gene transfer clinical trials demonstrated transgene-derived FVIII:C is ~2-fold higher by one-stage clotting assay (OSA) than chromogenic assay (CSA). Plasma…Factor XII heavy chain structure
Background: Human Factor XII is a zymogen form of coagulation protease factor XIIa which plays an important role in the initiation of intrinsic pathway of…“Off-the-shelf” cryopreserved platelets for the detection of HIT and VITT antibodies
Background: Heparin-induced thrombocytopenia (HIT) and vaccine-induced immune thrombotic thrombocytopenia (VITT) are life-threatening disorders characterized by anti-Platelet Factor 4 (PF4) antibodies. Tests such as the PF4-polyanion…Complement Receptors Mediate Binding of Heparin-Induced Thrombocytopenia Immune Complexes to Fc Receptor Bearing Cells
Background: Heparin-induced thrombocytopenia (HIT) is a thrombotic disorder caused by antibodies to platelet factor 4 (PF4)/heparin complexes that activate white blood cells (WBCs) via Fcγ…
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