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FVIII, factor VIII

Recombinant Factor VIII versus plasma derived Factor VIII in the management of haemophilia A: an examination of the costs and consequences

Recombinant Factor VIII versus plasma derived Factor VIII in the management of haemophilia A: an examination of the costs and consequences

... expected FVIII expenditure per purchasing district, based on a population of 500,000, using a prevalence rate of 1 case per 10,000 ...average FVIII consumption level per patient per year, reported by ...

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Adherence to treatment regimen and bleeding rates in a prospective cohort of youth and young adults on low-dose daily prophylaxis for severe hemophilia A

Adherence to treatment regimen and bleeding rates in a prospective cohort of youth and young adults on low-dose daily prophylaxis for severe hemophilia A

... anti-hemophilic factor replacement) is recommended as a standard of care by organizations such as the World Federation of Hemophilia, for treating severe hemophilia ...plasma factor VIII ...

6

Nanocapsule delivered Sleeping Beauty mediates therapeutic Factor VIII expression in liver sinusoidal endothelial cells of hemophilia A mice

Nanocapsule delivered Sleeping Beauty mediates therapeutic Factor VIII expression in liver sinusoidal endothelial cells of hemophilia A mice

... cis FVIII SB-Tns mediate long-term therapeutic expression in knockout hemophilia A ...initiation factor 4A1 promoter (IF) driving SB transposase version 10 (SB10) was constructed to express the BΔcFVIII ...

15

Hemophilia A gene therapy via intraosseous delivery of factor VIII-lentiviral vectors

Hemophilia A gene therapy via intraosseous delivery of factor VIII-lentiviral vectors

... of factor VIII (FVIII; abbreviated as F8 in constructs) is costly, inconvenient, and incompletely ...anti-factor VIII immune ...anti-factor VIII antibody formation is ...

7

Observational study of coagulation activation in early breast cancer: development of a prognostic model based on data from the real world setting

Observational study of coagulation activation in early breast cancer: development of a prognostic model based on data from the real world setting

... tissue factor (TF) pathway, DD levels beyond the 90th percentile were associated with cancer status, with results being not specific to the different sub- sets of patients as defined by hormone receptor (HR) and ...

9

Rituximab for managing acquired hemophilia A in a case of chronic neutrophilic leukemia with the JAK2 kinase V617F mutation

Rituximab for managing acquired hemophilia A in a case of chronic neutrophilic leukemia with the JAK2 kinase <em>V617F </em>mutation

... Case report: An 80-year-old Japanese male was diagnosed with acquired hemophilia A. He had compartment-like symptoms due to soft tissue hemorrhage in his left forearm and right lower extremity. A blood examination showed ...

5

Regulation of Adamts13 Function in Hemostasis by Cofactor and Substrate Exosite interactions

Regulation of Adamts13 Function in Hemostasis by Cofactor and Substrate Exosite interactions

... Willebrand factor (VWF) by ADAMTS13 is enhanced by clotting factor VIII ...in FVIII binding may be compromised in proteolytic cleavage by ADAMTS13 in the presence of ...of FVIII and ...

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Generic and disease-specific quality of life among youth and young men with Hemophilia in Canada

Generic and disease-specific quality of life among youth and young men with Hemophilia in Canada

... coagulation factor VIII (FVIII) and lifelong bleeding manifestations, particularly repeated hemarthroses leading to permanent joint ...intensive factor replacement therapy is administered ...

9

Localization of the binding regions of a murine monoclonal anti factor VIII antibody and a human anti factor VIII alloantibody, both of which inhibit factor VIII procoagulant activity, to amino acid residues threonine351 serine365 of the factor VIII heavy

Localization of the binding regions of a murine monoclonal anti factor VIII antibody and a human anti factor VIII alloantibody, both of which inhibit factor VIII procoagulant activity, to amino acid residues threonine351 serine365 of the factor VIII heavy chain

... anti-factor VIII (FVIII) inhibitory antibody (C5) to amino acid residues Thr351-Ser365 of the thrombin- generated 54-kD fragment of the heavy chain of ...Synthetic FVIII peptides were examined ...

7

Prevalence of factor VIII inhibitors in haemophilia a patients who received factor VIII therapy

Prevalence of factor VIII inhibitors in haemophilia a patients who received factor VIII therapy

... to factor concentrates While it is known that most inhibitors develop within the first 10-20 exposure days ...to FVIII during the first 6 months of life was associated with a higher incidence of inhibitor ...

120

Effective prophylaxis with rFVIIa in young haemophiliacs with inhibitors using a schedule similar to FVIII prophylaxis in non inhibitor patients

Effective prophylaxis with rFVIIa in young haemophiliacs with inhibitors using a schedule similar to FVIII prophylaxis in non inhibitor patients

... Haemorrhages and haemarthoses per year were re- duced during the prophylaxis period with rFVIIa. How- ever, it is not justifiable to compare the pre-prophylactic periods with the prophylaxis period because the risk of ...

7

Many factor VIII products available in the treatment of hemophilia A: an embarrassment of riches?

Many factor VIII products available in the treatment of hemophilia A: an embarrassment of riches?

... endogenous FVIII levels are > 5%, moderate when between 1% and 5%, and severe when < ...replacement factor first started with the use of fresh frozen plasma in the 1950s followed by cryoprecipitate in ...

7

Demonstration and characterization of specific binding sites for factor VIII/von Willebrand factor on human platelets

Demonstration and characterization of specific binding sites for factor VIII/von Willebrand factor on human platelets

... concentration. The optimal pH range for binding is from 7.0 to 7.5. At a concentration of 2 mM, EGTA inhibits 86% of the binding; however, 20 mM of Ca++, Mg++, or EDTA have little effect. Binding sites for ...

10

Elevated coagulation factor levels affect the tissue factor-threshold in thrombin generation

Elevated coagulation factor levels affect the tissue factor-threshold in thrombin generation

... proteins factor VIII (FVIII) and von Willebrand factor (VWF) are associated with a moderate to high risk for VTE ...with FVIII levels >150 IU/dL have a 6-fold increased risk for VTE ...

22

Factor VIII East Hartford (arginine 1689 to cysteine) has procoagulant activity when separated from von Willebrand factor

Factor VIII East Hartford (arginine 1689 to cysteine) has procoagulant activity when separated from von Willebrand factor

... of FVIII-EH plasma with penicillamine or DTT causes a five- to sixfold increase in FVIII-EH VIII:C, at 80 and 1 mM, ...no FVIII-EH light chain cleavage when thrombin is added in the presence of ...

7

Acquired Factor VIII Inhibitor in Juvenile Rheumatoid Arthritis

Acquired Factor VIII Inhibitor in Juvenile Rheumatoid Arthritis

... Acquired circulating inhibitor to factor VIII (FVIII) has been reported in both hemophiliacs and nonhe- mophiliac&.. FVIII inhibitor occurs in 10% to 20% of patients with severe, cla[r] ...

6

Hereditary combined deficiency of the vitamin K-dependent clotting factors

Hereditary combined deficiency of the vitamin K-dependent clotting factors

... mark) is a powerful drug with a short half-life (< 4 h) belonging to the so called "by-passing agents". It is indi- cated for the treatment of haemophilia complicated by the presence of anti-factor ...

8

Donor antigen-primed regulatory T cells permit liver regeneration and phenotype correction in hemophilia A mouse by allogeneic bone marrow stem cells

Donor antigen-primed regulatory T cells permit liver regeneration and phenotype correction in hemophilia A mouse by allogeneic bone marrow stem cells

... which factor VIII (FVIII) is inad- equately ...humans, FVIII is found to be synthesized by liver sinusoidal endothelial cells (LSECs) ...plasma FVIII [2, ...

16

Making plasma derived medicines available

Making plasma derived medicines available

... Decision makers of national heath care system should decide whether to use the available resources for importation of PDM or investing on developing infrastructure for local production of such medicines. Impor- tation of ...

9

Adeno-Associated Viral Vector-Driven Expression of Coagulation Proteins for Treatment of Hemophilias and Cancer

Adeno-Associated Viral Vector-Driven Expression of Coagulation Proteins for Treatment of Hemophilias and Cancer

... of FVIII or ...of FVIII 22 ...circulating FVIII or FIX, depending on the affected gene, in their bloodstream (described as negative for cross reactive material; ...

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