von Willebrand Disease (von Willebrand's Disease)

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1650 relevant articles (61 outcomes, 154 trials/studies) found for this Disease

Description: Group of hemorrhagic disorders in which the von Willebrand factor (Factor VIII-related antigen) is either quantitatively or qualitatively abnormal. They are usually inherited as an autosomal dominant trait though rare kindreds are autosomal recessive. Symptoms vary depending on severity and disease type but may include prolonged bleeding time, deficiency of factor VIII, and impaired platelet adhesion.

Also Known As:

von Willebrand's Disease; Angiohemophilias; Disease, von Willebrand; Disease, von Willebrand's; Hemophilias, Vascular; Vascular Hemophilia; Vascular Hemophilias; von Willebrands Disease; Angiohemophilia; Hemophilia, Vascular

Relationship Network

Disease Context: Research Results

Hemic and Lymphatic Diseases

Hematologic Diseases: 1480

Hemorrhagic Disorders: 456

von Willebrand Disease: 1650

Blood Coagulation Disorders: 88

Inherited Blood Coagulation Disorders: 34

von Willebrand Disease: 1650

Coagulation Protein Disorders

von Willebrand Disease: 1650

Blood Platelet Disorders: 78

von Willebrand Disease: 1650

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 449

Inborn Genetic Diseases: 578

Inherited Blood Coagulation Disorders: 34

von Willebrand Disease: 1650

Related Diseases

1.	Hemophilia A (Haemophilia)
2.	Hemorrhage
3.	Thrombocytopenia (Thrombopenia)
4.	Angiodysplasia
5.	Liver Diseases (Liver Disease)

Experts

1.	Federici, A B: 15 articles (05/2008 - 03/2000)
2.	Meyer, D: 13 articles (05/2008 - 01/2000)
3.	Mazurier, Claudine: 10 articles (05/2008 - 02/2003)
4.	Castaman, G: 10 articles (05/2008 - 01/2000)
5.	Rodeghiero, F: 9 articles (05/2008 - 01/2000)
6.	Mazurier, C: 9 articles (05/2008 - 02/2001)
7.	Mannucci, P M: 8 articles (05/2008 - 01/2000)
8.	Meyer, Dominique: 7 articles (05/2008 - 02/2003)
9.	Goudemand, Jenny: 7 articles (05/2008 - 03/2004)
10.	Budde, Ulrich: 7 articles (05/2008 - 06/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to von Willebrand Disease:

1.	von Willebrand FactorIBA 07/01/2007 - "BACKGROUND: While plasma-derived concentrates containing large amounts of von Willebrand factor (VWF) are effective in treating von Willebrand disease (VWD), optimal dosing remains to be fully characterized. " 01/01/1995 - "The definition of the molecular basis of von Willebrand's disease has helped clarify the structure of von Willebrand factor as well as its essential role in platelet function, particularly under haemodynamic conditions of high shear stress. " 02/01/2006 - "Increased clearance of von Willebrand factor (VWF) is one of the main features of type Vicenza von Willebrand disease (VWD), a variant with plasma and platelet VWF level discrepancies and unusually large VWF multimers. " 02/01/2007 - "BACKGROUND: von Willebrand disease (VWD) type 2 is associated with mutations in von Willebrand factor (VWF) that affect its secretion, multimeric pattern, affinity for platelet receptors and clearance of the protein. " 01/01/2006 - "The efficacy of the two von Willebrand factor (vWF) intron 40 variable number of tandem repeat (VNTR) markers, vWF1 and vWF2, in the genetic diagnosis of von Willebrand disease (vWD) in Indian patients was studied. " Order ALL the reference details at left...
2.	Factor VIII (Coagulation Factor VIII)IBA 07/15/1983 - "The efficacy of high purity preparations of factor VIII complex are usually lacking effect on the bleeding time in von Willebrand's disease. " 03/01/1989 - "The authors describe a patient with von Willebrand's disease in whom pregnancy itself improved factor VIII activity, enabling performance of an epidural block for labor and delivery." 09/01/1983 - "Recent advances in characterizing and measuring the factor VIII complex have greatly improved the diagnosis of the variant forms of von Willebrand's disease. " 08/01/2008 - "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. " 02/01/1998 - "[Ultrapure factor VIII concentrate with double virus inactivation: study of pharmacokinetics in von Willebrand's disease patients]" Order ALL the reference details at left...
3.	Deamino Arginine Vasopressin (Desmopressin)FDA LinkGeneric 03/01/2009 - "Until the mid-80s, cryoprecipitate has been the mainstay of treatment of patients with von Willebrand disease who were unresponsive to desmopressin. " 04/01/2005 - "Until the mid 1980s, cryoprecipitate had been the mainstay of treatment of patients with von Willebrand disease (VWD) who were unresponsive to desmopressin. " 12/01/1994 - "Re-evaluation of the therapeutic efficacy of DDAVP in type IIB von Willebrand's disease." 03/01/2002 - "Efficacy of desmopressin as surgical prophylaxis in patients with acquired von Willebrand disease undergoing thyroid surgery." 04/01/2008 - "Response to desmopressin is influenced by the genotype and phenotype in type 1 von Willebrand disease (VWD): results from the European Study MCMDM-1VWD." Order ALL the reference details at left...
4.	AntigensIBA 06/01/1978 - "This study reports a second von Willebrand's disease antigen (vWagII), distinct from VIIIag, that is also deficient in the platelets and plasma of patients with severe von Willebrand's disease. " 01/01/2003 - "Results obtained with the flow cytometric method on samples from two patients with von Willebrand disease 2B were lower than those obtained with the antigen method in accordance with the diagnosis. " 01/01/2003 - "RESULTS: In samples from healthy controls and from patients with von Willebrand disease type 1, the flow cytometry assay showed good correlation with the VWF:RCo assay (r2 = 0.69) and the VWF antigen assays (r2 = 0.83), which was better than the correlation between the VWF:RCo assay and VWF antigen assays (r2 = 0.72). " 03/01/1997 - "Injection of the anti-FVIII/vWF inhibitor plasma into mice caused a decrease in vWF antigen, in some animals with a complete loss of vWF multimers comparable to severe von Willebrand disease. " 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. " Order ALL the reference details at left...
5.	CollagenIBA 10/15/2009 - "We propose that isolated collagen-binding defects are classified as a distinct subtype of von Willebrand disease." 01/01/2007 - "Performance and utility of a cost-effective collagen-binding assay for the laboratory diagnosis of 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." 01/01/2004 - "OBJECTIVE: To explore the application of collagen-binding assay for von Willebrand disease (vWD). " Order ALL the reference details at left...
6.	Vasopressins (Vasopressin)IBA 02/01/2005 - "Cerebral arterial thrombosis in a young woman following vasopressin for von Willebrand's disease." 06/01/1984 - "The subcutaneous administration of the vasopressin analogue 1-desamino-8-D-arginine vasopressin in patients with von Willebrand's disease and hemophilia." 11/26/1980 - "The use of 1 deamino-8-D-arginine vasopressin (DDVAP), is reported in seven patients with von Willebrand's disease and mild haemophilia undergoing elective surgery. " 06/01/1982 - "Studies of the blood plasminogen activator induced by 1-desamino-8-D-arginine vasopressin with observations in von Willebrand's disease." 01/01/1998 - "The synthetic vasopressin analogue, desmopressin (DDAVP), has reduced the need for transfusions in most of the mild forms of von Willebrand disease but DDAVP is ineffective in type 3 and in other severe cases of types 1 and 2 von Willebrand disease. " Order ALL the reference details at left...
7.	Fibrinogen (Factor I)IBA 01/01/2000 - "In countries lacking the economic resources to obtain virally inactivated concentrates, MB-cryoprecipitates could be useful in von Willebrand's disease and fibrinogen and FXIII deficiencies. " 02/01/1985 - "The antibody bound with equal affinity in solid phase radioimmunoassays to fibrinogens isolated from both normal patients and patients with von Willebrand disease. " 09/01/2002 - "It will be of particular interest to determine the efficacy of rVIIa in conditions such as severe thrombocytopenia, severe von Willebrand disease, severe defects in platelet activation, and severe deficiencies of factors V, X, II, and fibrinogen in which effectiveness would seem to be unlikely based on our current understanding of mechanisms of action of rVIIa." 11/01/2003 - "Deep vein thrombosis after orthopedic surgery in a patient with type 1 von Willebrand disease and mutations in the MTHFR and beta-fibrinogen genes." 11/15/2002 - "The impaired thrombus growth in AF was only partially corrected by the addition of purified fibrinogen to AF blood, whereas the addition of purified VWF to blood of severe von Willebrand disease (VWD) completely normalized the defective thrombus growth in this disease. " Order ALL the reference details at left...
8.	Immunoglobulins (Immunoglobulin)IBA 11/01/1997 - "Acquired type 2a von Willebrand's disease: response to immunoglobulin infusion." 06/01/2005 - "Recently, the hematologic literature has reported continuous infusion of factor concentrates and intravenous immunoglobulin as an improved therapy for active bleeding and prophylaxis in patients who are anticipating surgery with congenital von Willebrand disease. " 10/01/2007 - "OBJECTIVE: To describe a patient with common variable immunodeficiency (CVID) and von Willebrand disease who tolerated immunoglobulin replacement via the subcutaneous route. " 05/01/1995 - "High-dose intravenous immunoglobulin delays clearance of von Willebrand factor in acquired von Willebrand disease." 06/01/2005 - "Acquired von Willebrand disease: management of labor and delivery with intravenous dexamethasone, continuous factor concentrate, and immunoglobulin infusion." Order ALL the reference details at left...
9.	alphanateIBA 01/01/1998 - "Products thought to be effective in von Willebrand disease included Haemate P, Facteur von Willebrand, Alphanate, and the UK's BPL '8Y'. " 03/01/2008 - "Efficacy of factor VIII/von Willebrand factor concentrate Alphanate in preventing excessive bleeding during surgery in subjects with von Willebrand disease." Order ALL the reference details at left...
10.	RistocetinIBA 11/01/2007 - "Differential identification of a rare form of platelet-type (pseudo-) von Willebrand disease (VWD) from Type 2B VWD using a simplified ristocetin-induced-platelet-agglutination mixing assay and confirmed by genetic analysis." 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. " 06/01/2005 - "Addition of EMP to plasma from severe von Willebrand disease restored his ristocetin-induced platelet aggregation. " 01/01/2002 - "Six of the 29 children (20.7%) had decreased values of ristocetin and cofactor activity and were considered to have acquired von Willebrand's disease. " 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. " Order ALL the reference details at left...

Therapies and Procedures

1.	Plasma Exchange 05/01/1987 - "The efficacy of intensive plasma exchange in acquired von Willebrand's disease." 10/01/1989 - "Treatment of von Willebrand's disease and hypofibrinogenemia with single donor cryoprecipitate from plasma exchange donation." 05/01/1987 - "The current case demonstrates that the clinical and laboratory abnormalities in a patient with acquired von Willebrand's disease can be corrected completely by plasma exchange; it is recommended therefore that plasma exchange be considered as a mode of therapy in symptomatic patients with this disorder." 05/01/1987 - "This study describes the response to therapeutic plasma exchange in a 60-year-old man with Waldenström's macroglobulinemia who developed a clinically severe bleeding disorder with laboratory features characteristic of acquired von Willebrand's disease. " 11/22/1980 - "A sixth patient with von Willebrand's disease and a factor VIII clotting activity inhibitor was successfully prepared for operation using plasma exchange. " Order ALL the reference details at left...
2.	Injections 05/01/2002 - "The increasing clinical experience with CI used in haemophilia A and B and von Willebrand disease has proven the advantages of this mode of replacement therapy, providing improved margins of efficacy and safety in various clinical settings requiring the maintenance of haemostatic levels over prolonged periods, as well as reduction in treatment cost compared with the traditional therapy via intermittent injections of coagulation factors.Keywords" Order ALL the reference details at left...
3.	Anesthesia 06/01/2007 - "[Von Willebrand's disease and anesthesia.]" 05/01/2007 - "The indications for neuraxial anesthesia with regard to type 1 von Willebrand disease are reviewed." 06/01/2003 - "[Anesthesia for septoplasty and turbinectomy in von Willebrand disease patient: case report.]" 05/01/1981 - "[Anesthesia for a patient with von Willebrand's disease (author's transl)]" 07/01/2009 - "No consensus exists about the use of neuraxial analgesia or anesthesia in patients with von Willebrand disease. " Order ALL the reference details at left...
4.	Transplants (Transplant) 04/01/2007 - "We describe a patient with type 2N von Willebrand's disease scheduled for elective coronary artery bypass graft for severe three-vessel coronary artery disease with involvement of the left main stem. " 04/01/2007 - "Peri-operative management of an adult patient with type 2N von Willebrand's disease scheduled for coronary artery bypass graft." 01/01/1993 - "Management of a patient with type IIC von Willebrand's disease during coronary artery bypass graft surgery." 02/01/2003 - "OBJECTIVE: Deposition of von Willebrand factor (vWF) is increased in hyperplastic intima of grafts, vWF levels are elevated in patients with cardiovascular diseases, and there is resistance to progression of atherosclerosis in pigs with von Willebrand disease. " 08/01/1977 - " Presently, there is no means of inducting von Willebrand disease; however, evidence on the preventability of atherosclerosis could be gleaned from controlled, long-term trials of anticoagulants and platelet inhibitors in coronary by-pass graft recipients." Order ALL the reference details at left...
5.	Endometrial Ablation Techniques 09/01/2004 - "Endometrial ablation for von Willebrand disease-related menorrhagia--experience with seven cases." 06/01/2001 - "STUDY DESIGN: In a pilot clinical study, endometrial ablation was performed under intravenous thiopental anesthesia on five women suffering from hypermenorrhea caused by aplastic anemia (3), von Willebrand's disease (1) and a submucosal myoma (1) using a microwave tissue coagulator at 2.45 GHz. " Order ALL the reference details at left...

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