|3.||von Willebrand Diseases (von Willebrand's Disease)
|4.||Blood Platelet Disorders (Thrombocytopathy)
|5.||Hemophilia A (Haemophilia)
|1.||Federici, A B: 9 articles (02/2010 - 01/2000)|
|2.||Meyer, D: 8 articles (04/2007 - 06/2000)|
|3.||Mazurier, Claudine: 7 articles (09/2006 - 06/2002)|
|4.||Fressinaud, E: 6 articles (04/2007 - 06/2001)|
|5.||Mazurier, C: 6 articles (12/2004 - 12/2000)|
|6.||Fressinaud, Edith: 5 articles (12/2013 - 02/2003)|
|7.||Favaloro, Emmanuel J: 5 articles (01/2011 - 08/2007)|
|8.||Michiels, Jan Jacques: 5 articles (06/2010 - 06/2004)|
|9.||Canciani, M T: 5 articles (02/2010 - 12/2000)|
|10.||Mannucci, P M: 5 articles (02/2007 - 01/2000)|
|1.||von Willebrand FactorIBA
08/01/2005 - "The Y/C1584 mutation of von Willebrand factor in type 2M von Willebrand disease: frequency and clearance of von Willebrand factor."
04/01/1996 - "Analysis of Arg834Gln and Val902Glu type 2A von Willebrand disease mutations: studies with recombinant von Willebrand factor and correlation with patient characteristics."
02/15/1989 - "In the present studies we report that this high molecular weight endothelial cell-derived von Willebrand factor, when incubated with granulocyte lysates, was cleaved into a series of multimers indistinguishable from those seen in normal plasma (or type II von Willebrand disease). "
09/01/1987 - "Studies were conducted to characterize the biosynthesis of von Willebrand factor (vWf) by cultured endothelial cells (EC) derived from the umbilical vein of a patient with type IIA von Willebrand disease. "
05/01/2015 - "Enhanced von Willebrand factor (VWF) clearance is important in the etiology of type 1 and type 2 von Willebrand disease (VWD). "
|2.||Deamino Arginine Vasopressin (Desmopressin)FDA LinkGeneric
03/15/2004 - "Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter European study."
09/01/2009 - "However, although occasional correction of closure times was observed in patients with type 2A or type 2M von Willebrand disease, these did not in general normalize PFA-100 closure times either with desmopressin or factor concentrate therapy. "
05/01/2009 - "We have prospectively evaluated the biologic response to desmopressin (DDAVP) in 28 children with type 2 von Willebrand disease (VWD) in correlation with the phenotype and the molecular defect of VWF. "
05/01/2009 - "Response to DDAVP in children with von Willebrand disease type 2."
05/01/2008 - "Biologic response to subcutaneous and intranasal therapy with desmopressin in a large Amish kindred with Type 2M von Willebrand disease."
|3.||Platelet Glycoprotein GPIb-IX Complex (Glycoprotein Ib)IBA
04/01/1992 - "We and others have recently identified a panel of missense mutations clustered in the platelet glycoprotein Ib binding domain of vWF from patients with type IIB von Willebrand disease. "
01/01/2015 - "Thrombocytopenia and increased platelet clearance observed in von Willebrand disease-type 2B (VWD-2B) may be explained by platelet apoptosis triggered by the constitutive binding of VWF to its receptor, glycoprotein Ib (GPIb). "
12/20/1993 - "Effect of type IIB von Willebrand disease mutation Arg(545)Cys on platelet glycoprotein Ib binding--studies with recombinant von Willebrand factor."
12/01/2013 - "von Willebrand disease type 2B (vWD-type 2B) is characterized by gain-of-function mutations in von Willebrand factor (vWF) that enhance its binding to the glycoprotein Ib-IX-V complex on platelets. "
06/01/2006 - "Type 2B von Willebrand disease (VWD) is characterised by an increased affinity of von Willebrand factor (VWF) for its platelet receptor glycoprotein Ib (GPIb). "
01/01/2011 - "However, after correction for the VWF antigen levels, by calculation of FVIII binding/VWF antigen ratio, only the patients with known von Willebrand disease type 2N (n = 4) had clearly abnormal results. "
09/01/1996 - "vWF containing the S1613P mutation found in some type 2A von Willebrand disease (vWD) patients was observed to undergo proteolysis in vivo resulting in a reduction of high molecular weight vWF and concomitant appearance of rapidly-migrating satellite species, although the overall clearance rate of vWF antigen was similar to wild type vWF. "
11/01/2009 - "Type 2M von Willebrand disease (VWD) is characterized by a qualitative defect in von Willebrand factor (VWF) and diagnosed by a disproportionate decrease in VWF ristocetin cofactor activity (VWF:RCo) as compared with VWF antigen (VWF:Ag). "
11/01/1980 - "The Factor VIII antigen molecules in the plasma of patients with classical type 1 and variant type 2A von Willebrand disease were compared to the Factor VIII antigen molecules in normal plasma. "
09/01/2007 - "In order to better characterize the genotype-phenotype correlation in type 2M von Willebrand disease (VWD), we sequenced the coding region for the mature subunit of the von Willebrand factor (VWF) gene (exons 18-52, including exon/intron boundaries) in 16 index cases originally submitted to the Canadian Type 1 VWD Study as type 1 VWD, but reclassified as type 2M VWD on the basis of phenotype (excessive mucocutaneous bleeding and von Willebrand factor: antigen (VWF:Ag) and/or von Willebrand factor: ristocetin cofactor (VWF:RCo) between 0.05 and 0.50 IU mL(-1) on at least two occasions and RCo/Ag ratio < 0.6 and no loss of high molecular weight multimers). "
|5.||Heparin (Liquaemin)FDA LinkGeneric
12/01/2001 - "The aim of our study was to characterise heparin-binding properties of mutated von Willebrand factor (VWF) in 24 patients plasmas with type 2 von Willebrand disease (VWD). "
12/01/2001 - "Defect of heparin binding in plasma and recombinant von Willebrand factor with type 2 von Willebrand disease mutations."
10/15/1992 - "Recently, missense mutations that cause type IIB von Willebrand disease (vWD) were described, clustered within a disulfide loop in the A1 domain of vWF that has binding sites for GPIb, collagen, and heparin. "
10/15/1992 - "A type IIB von Willebrand disease mutation affects binding to glycoprotein Ib but not to collagen or heparin."
09/01/2010 - "A randomised pilot trial of the anti-von Willebrand factor aptamer ARC1779 in patients with type 2b von Willebrand disease."
08/01/2012 - "The anti-von Willebrand factor aptamer ARC1779 increases von Willebrand factor levels and platelet counts in patients with type 2B von Willebrand disease."
08/01/2012 - "Blockade of hyperactive von Willebrand factor (VWF) by ARC1779 blunted the platelet drop induced by desmopressin in patients with type 2B von Willebrand disease (VWD). "
|7.||von Willebrand factor drug combination factor VIIIIBA
09/01/1979 - "The transfusion study of this patient with acquired von Willebrand disease type II (variant of von Willebrand disease) serves to emphasize the relationship between polydispersity of Factor VIII/von Willebrand Factor and functional heterogeneity."
06/01/2004 - "Bleeding prophylaxis for major surgery in patients with type 2 von Willebrand disease with an intermediate purity factor VIII-von Willebrand factor concentrate (Haemate-P)."
01/01/2014 - "VWF multimer analysis showed a loss of high-molecular-weight multimers and his plasma aggregated normal platelets under low ristocetin concentration, consistent with type 2B von Willebrand disease (VWD). "
12/01/2010 - "Prospective study of low-dose ristocetin-induced platelet aggregation to identify type 2B von Willebrand disease (VWD) and platelet-type VWD in children."
07/01/2007 - "Furthermore, in contrast to plasma VWF, the ultra-large (UL)VWF multimers or a recombinant VWF-A1A2A3 polypeptide containing a gain-of-function mutation (R1308 L) of type 2B von Willebrand disease bound to the A2 domain polypeptide without the need for ristocetin. "
06/15/2000 - "We report on 9 fully multimerized recombinant vWFs (rvWFs) expressing type 2M or type 2B von Willebrand disease (vWD) mutations, characterized respectively by a decreased or increased binding of vWF to GPIb in the presence of ristocetin. "
08/01/1996 - "The variant molecules resulting in type 2B von Willebrand disease can sustain binding to platelets at subnormal levels of ristocetin. "
|9.||Factor VIII (Coagulation Factor VIII)IBA
12/15/2000 - "Both her parents had normal values for factor VIII activity, and von Willebrand disease type 2N was excluded. "
06/01/1998 - "Factor VIII also bound to platelets obtained from a patient with Type 2N von Willebrand disease, although in this case the binding was reduced to approximately 50% of that seen with control platelets. "
08/01/2008 - "Type 2N von Willebrand disease (vWD) can be confused with hemophilia A due to decreased factor VIII levels and a bleeding tendency, and differential diagnosis is of importance for providing the optimal treatment and genetic counseling. "
01/01/2011 - "Interaction of factor VIII and von Willebrand factor and the identification of type 2N von Willebrand disease."
05/01/2009 - "Laboratory diagnosis of von Willebrand disease type 2N (VWD2N) is based on costly mutation analysis or in vitro measurement of the ability of plasma von Willebrand factor (VWF) to bind exogenous factor VIII (FVIII); however, the VWF-FVIII binding activity assay is complex and not widely used. "
07/01/2013 - "Variability in platelet- and collagen-binding defects in type 2M von Willebrand disease."
02/01/2006 - "The interaction of von Willebrand factor-A1 domain with collagen: mutation G1324S (type 2M von Willebrand disease) impairs the conformational change in A1 domain induced by collagen."
02/01/2006 - "Development of a collagen-binding activity assay as a screening test for type II von Willebrand disease in dogs."
07/01/2013 - "Type 2M von Willebrand disease (VWD) includes qualitative defects in von Willebrand factor (VWF) function, with normal multimer distribution but a defect in VWF activity with respect to platelet or collagen binding. "
09/01/2006 - "von Willebrand factor collagen binding assay in von Willebrand disease type 2A, 2B, and 2M."
|3.||Cesarean Section (Caesarean Section)