|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Among the remaining 9504 patients still being followed up, 5748 (60.5%) had haemophilia A, 1300 (13.7%) haemophilia B, 1980 (20.8%) von Willebrand Disease while 476 (5.0%) had another clotting factor deficiency (Factor I, II, V, combined V and VIII, VII, X, XI and XIII).
|
30515664 |
2019 |
|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
The Course of von Willebrand Factor and Factor VIII Activity in Patients with von Willebrand Disease during Pregnancy.
|
31085919 |
2019 |
|
von Willebrand Disease
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
There are a number of other disorders that are now typically treated in these comprehensive care centers including von Willebrand disease (VWD), rare factor deficiencies (I, II, V, V & VIII, VII, X, XI and XIII), and inherited platelet function disorders.
|
31427262 |
2019 |
|
von Willebrand Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
The three main screening tests used in the diagnosis of VWD include von Willebrand Factor (VWF) antigen, platelet-dependent VWF activity, and factor VIII activity.
|
30064913 |
2018 |
|
von Willebrand Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Long-term outcome after joint bleeds in von Willebrand disease (VWD) (von Willebrand factor activity ≤ 30 IU/dL) could differ from moderate or severe haemophilia A (HA) (factor VIII [FVIII] 1-5 IU/dL or FVIII < 1 IU/dL).
|
30273954 |
2018 |
|
von Willebrand Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
: Men and boys who present with bleeding associated with low factor VIII levels and normal von Willebrand studies are assumed to have hemophilia A until proven otherwise.
|
30036281 |
2018 |
|
von Willebrand Disease
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
A 30-question survey constituted the input to discuss (i) optimisation of the diagnostic workflow for vWD, (ii) the characteristics of patients who may benefit from secondary long-term prophylaxis (in particular with the purified von Willebrand factor concentrate with a low content of factor VIII), (iii) the key elements to consider when selecting a concentrate and (iv) the pre-operative and post-operative management of vWD patients.
|
29313798 |
2018 |
|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Haemate-P/Humate-P (Humate-P) is a pasteurized human plasma-derived concentrate containing both Factor VIII and von Willebrand factor for treatment of hemophilia A and von Willebrand disease (VWD).
|
28691218 |
2017 |
|
von Willebrand Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Cases with isolated prolonged APTT were tested for factors VIII and IX using factor assays This was followed by FXI:C level assessment in cases with normal FVIII and FIX levels. vWD was screened in patients with low FVIII levels.
|
28388959 |
2017 |
|
von Willebrand Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
In this issue of Blood, Sanders and coworkers define the pathophysiology of types 1, 2, and 3 von Willebrand disease (VWD) in the Willebrand in the Netherlands (WiN) study by using the ratios of von Willebrand factor propeptide (VWFpp) or factor VIII activity to VWF antigen.
|
25953977 |
2015 |
|
von Willebrand Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
In summary, rhIL-11 increases VWF levels in two of four DDAVP-unresponsive or allergic VWD and F.VIII levels in four of five mild or moderate haemophilia A subjects, suggesting its potential use in treatment of these disorders.
|
23238591 |
2013 |
|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
These inherited disorders include factor I, V, VII, X, XI, XIII deficiency, combined factor VII and X deficiency, combined factor V and VIII deficiency, all platelet disorders and von Willebrand disease (VWD) type III.
|
21451397 |
2011 |
|
von Willebrand Disease
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Women with von Willebrand's disease and R1205H and C1130F mutations (17 pregnancies in 12 women) had only a slight increase of factor VIII and von Willebrand factor during pregnancy while their response to desmopressin was marked but short-lived.
|
19951969 |
2010 |
|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
The safety of plasma-derived von Willebrand/factor VIII concentrates in the management of inherited von Willebrand disease.
|
19309248 |
2009 |
|
von Willebrand Disease
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Factor VIII-von Willebrand factor binding defects in autosomal recessive von Willebrand disease type Normandy and in mild hemophilia A. New insights into factor VIII-von Willebrand factor interactions.
|
19506355 |
2009 |
|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Undetected factor VIII in a patient with type 3 von Willebrands disease mistaken as severe haemophilia A.
|
19563498 |
2009 |
|
von Willebrand Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Genetic studies were performed in a patient with a phenotype of von Willebrand disease (VWD) characterized by very low plasma factor VIII and VWF levels and a VWF consisting of only a dimeric band and total absence of all multimers in plasma.
|
18690339 |
2008 |
|
von Willebrand Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
The aim of treatment of von Willebrand disease (VWD) is to correct the dual defect of hemostasis (i.e., the abnormal platelet adhesion due to reduced and/or dysfunctional von Willebrand factor [VWF] and the abnormal coagulation expressed by low levels of factor [F] VIII).
|
16977571 |
2006 |
|
von Willebrand Disease
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Recombinant expression of mutations causing von Willebrand disease type Normandy: characterization of a combined defect of factor VIII binding and multimerization.
|
15213842 |
2004 |
|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Interleukin 11 significantly increases plasma von Willebrand factor and factor VIII in wild type and von Willebrand disease mouse models.
|
11154224 |
2001 |
|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
We used a murine model of severe von Willebrand disease (vWF knockout mice) to study the effect of a recombinant vWF/pro-vWF preparation (rpvWF) on factor VIII survival and to investigate whether low-density lipoprotein receptor-related protein (LRP) might be involved in the in vivo clearance of factor VIII in the absence of vWF. vWF-deficient mice received 70 U/kg rpvWF in the first series of experiments, and in a second series, 80 mg/kg receptor-associated protein (RAP) as a recombinant fusion protein to block the action of LRP.
|
10688827 |
2000 |
|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Traits that exhibited significant genetic correlations with thrombosis included levels of several coagulation factors (factors VII, VIII, IX, XI, XII, and von Willebrand), tissue plasminogen activator, homocysteine, and the activated protein C ratio.
|
11038326 |
2000 |
|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
After infusion, a prolonged increase of FVIII (> 48 h) was observed, suggesting that human recombinant vWF is able to bind and to stabilize porcine factor VIII and that porcine vWD is a good model for studying such interactions.
|
9690808 |
1998 |
|
von Willebrand Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
The laboratory diagnosis of vWD and its several variants is made on the basis of immunologic and functional studies of vWF, factor VIII levels, and specialized electrophoretic analysis (multimer gels).
|
9046981 |
1997 |
|
von Willebrand Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
A test infusion with this agent is advisable in patients with severe vWD before considering treatment with VIII/vWF concentrates.
|
7833254 |
1995 |