Laboratory Diagnosis and Molecular Basis of Mild von Willebrand Disease Type 1

2009 ◽  
Vol 121 (2-3) ◽  
pp. 85-97 ◽  
Author(s):  
Jan Jacques Michiels ◽  
Zwi Berneman ◽  
Alain Gadisseur ◽  
Marc van der Planken ◽  
Wilfried Schroyens ◽  
...  
Keyword(s):  
Molecular Basis ◽  
Laboratory Diagnosis ◽  
Disease Type ◽  
Von Willebrand Disease ◽  
Von Willebrand

Recessive von Willebrand Disease Type 2 Normandy: Variable Expression of Mild Hemophilia and VWD Type 1

2009 ◽  
Vol 121 (2-3) ◽  
pp. 119-127 ◽  
Author(s):  
Jan Jacques Michiels ◽  
Alain Gadisseur ◽  
Inge Vangenegten ◽  
Wilfried Schroyens ◽  
Zwi Berneman
Keyword(s):  
Disease Type ◽  
Von Willebrand Disease ◽  
Variable Expression ◽  
Von Willebrand

Expression of angiogenic factors in the uteroplacental unit is altered at time of placentation in a porcine model of von Willebrand disease type 1

2019 ◽  
Vol 19 (4) ◽  
pp. 412-420
Author(s):  
Hanna Allerkamp ◽  
Stefanie Lehner ◽  
Mahnaz Ekhlasi-Hundrieser ◽  
Carsten Detering ◽  
Mario von Depka Prondzinski ◽  
...  
Keyword(s):  
Porcine Model ◽  
Disease Type ◽  
Angiogenic Factors ◽  
Von Willebrand Disease ◽  
Von Willebrand

Von Willebrand disease type 1 in a porcine model influences expression of integrin αVβ3, members of the VEGF/VEGFR-2 system and ANG/TIE ratios at the time of placentation

Placenta ◽  
2019 ◽  
Vol 83 ◽  
pp. e18
Author(s):  
Hanna Allerkamp ◽  
Stefanie Lehner ◽  
Carsten Detering ◽  
Mahnaz Ekhlasi-Hundrieser ◽  
Mario von Depka Prondzinski ◽  
...  
Keyword(s):  
Porcine Model ◽  
Integrin Αvβ3 ◽  
Disease Type ◽  
Von Willebrand Disease ◽  
Von Willebrand

Managing Patients with von Willebrand Disease Type 1, 2 and 3 with Desmopressin and von Willebrand Factor-Factor VIII Concentrate in Surgical Settings

2009 ◽  
Vol 121 (2-3) ◽  
pp. 167-176 ◽  
Author(s):  
Jan Jacques Michiels ◽  
Huub H.D.M. van Vliet ◽  
Zwi Berneman ◽  
Wilfried Schroyens ◽  
Alain Gadisseur
Keyword(s):  
Factor Viii ◽  
Von Willebrand Factor ◽  
Disease Type ◽  
Von Willebrand Disease ◽  
Von Willebrand ◽  
Willebrand Factor

scholarly journals Quantitative impact of using different criteria for the laboratory diagnosis of type 1 von Willebrand disease

2014 ◽  
Vol 12 (8) ◽  
pp. 1238-1243 ◽  
Author(s):  
T. Quiroga ◽  
M. Goycoolea ◽  
S. Belmont ◽  
O. Panes ◽  
E. Aranda ◽  
...  
Keyword(s):  
Laboratory Diagnosis ◽  
Von Willebrand Disease ◽  
Von Willebrand ◽  
Quantitative Impact

Desmopressin parenteral bei VWD 1, VWD 2A und Thrombozytopathie

2011 ◽  
Vol 31 (S 01) ◽  
pp. S29-S33 ◽  
Author(s):  
H. Pollmann ◽  
B. Siegmund
Keyword(s):  
Side Effects ◽  
Disease Type ◽  
Von Willebrand Disease ◽  
Occlusion Time ◽  
Almost All ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Ristocetin Cofactor

SummaryDesmopressin (DDAVP, Minirin® parenteral), which induces the release of von-Willebrand factor from endogenous stores, is indicated in von Willebrand disease type 1 (VWD 1). In the present study effectiveness of DDAVP was tested and side effects were recorded in patients with VWD 1, von Willebrand disease type 2 (VWD 2) or thrombocytopathy (TCP). Patients, methods Subjects were analysed prior to and after Minirin parenteral infusion (0.4 μg/kg body weight (b.w.) over 60 minutes) for partial thromboplastin time (PTT, seconds), ADP/epinephrine triggered plateletfunction analyzer (PFA-100) occlusion time (seconds), factor VIII activity (FVIII, %), VWF as ristocetin cofactor activity (VWF:RCo, %) and VWF antigen (VWF:Ag, %). Side effects of DDAVP during operative interventions were recorded per questionnaires by the patients. Results The mean ± standard deviation dose (n = 165 patients) of Minirin parenteral administered was 0.37 ± 0.02 μg/kg b.w., most often upcoming dental operations (57%) necessitated testing. Coagulation parameters of patients with VWD 1 or TCP normalised in almost all patients, but only in approximately 50% of patients with VWD 2 respectively. Appraisal of effectiveness of Minirin parenteral as good was 96% in case of VWD 1 and 95 % in case of TCP. During minor surgeries (n = 23) in 91% of the patients no complications and in 2 patients (9%) postoperative haemorrhages without need for further interventions occurred, but 83% of the patients reported adverse reactions in the questionnaires, although Minirin parenteral was well tolerated by all patients during DDAVP efficacy tests. Conclusion Desmopressin is well tolerated and affective in patients with VWD 1 and thrombocytopathy.

Molecular Basis of von Willebrand Disease Type IIC Miami

2002 ◽  
pp. 203-207
Author(s):  
R. Schneppenheim ◽  
T. Obser ◽  
J. U. Wieding ◽  
F. Bergmann ◽  
E. Drewke ◽  
...  
Keyword(s):  
Molecular Basis ◽  
Disease Type ◽  
Von Willebrand Disease ◽  
Von Willebrand

Laboratory Diagnosis of von Willebrand Disease

2009 ◽  
Vol 03 (01) ◽  
pp. 33 ◽  
Author(s):  
Muriel Meiring ◽  
Philip N Badenhorst ◽  
Mareli Kelderman ◽  
◽  
◽  
...  
Keyword(s):  
Factor Viii ◽  
Correct Diagnosis ◽  
Laboratory Diagnosis ◽  
Binding Activity ◽  
Von Willebrand Disease ◽  
Coagulant Activity ◽  
Low Sensitivity ◽  
Von Willebrand ◽  
Willebrand Factor

von Willebrand disease (VWD) is a bleeding disorder caused by either quantitative (type 1 and 3) or qualitative (type 2) defects of von Willebrand factor (VWF). No single available test provides appropriate information about the various functions of VWF, and the laboratory diagnosis of VWD is based on a panel of tests, including the measurement of factor VIII coagulant activity (FVIIIC), VWF antigen levels (VWF:Ag), VWF activity as measured by the ristocetin co-factor activity (VWF:RCo), the collagen-binding activity of VWF (VWF:CB), VWF multimer analysis, ristocetininduced platelet agglutination (RIPA), the factor-VIII-binding assay of plasma VWF and VWF propeptide levels. Due to the heterogeneity of VWF defects and the variables that interfere with VWF levels, a correct diagnosis of types and subtypes may sometimes be difficult, but is very important for therapy. Furthermore, the RCo assay and the RIPA test are based on platelet agglutination in reaction with the non-physiological antibiotic ristocetin. These tests also have low sensitivity and are difficult to standardise. Therefore, several analyses (tests) are required to diagnose VWD and it is important to be aware of the pitfalls to which these tests are subjected in terms of the diagnosis. In this article, the laboratory diagnosis of patients with type 1, 2A, 2B, 2M, 2N and 3 VWD will be explained by using a modified algorithm that was first proposed by the guidelines for diagnosis and treatment of VWD in Italy.

Sign in / Sign up

Export Citation Format

Share Document