Type 1 von Willebrand Disease

10/01/2017 - "Abnormalities in the biosynthetic pathway or increased clearance of plasma von Willebrand factor (VWF) are likely to contribute to decreased plasma VWF levels in inherited type 1 von Willebrand disease (VWD). "
08/01/2009 - "Expression of 14 von Willebrand factor mutations identified in patients with type 1 von Willebrand disease from the MCMDM-1VWD study."
04/01/2007 - "Impact of plasma von Willebrand factor levels in the diagnosis of type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1VWD)."
11/29/2023 - "The von Willebrand factor (VWF) gene exhibits a significant degree of sequence variability and the first VWF variant associated with type 1 von Willebrand disease (VWD), c.4751 A>G, p.Y1584C, was described in 2003. "
11/27/2023 - "Von Willebrand Factor (VWF) multiplex activity assay differentiation of type 1 von Willebrand Disease (VWD) and variant VWD."

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."
08/01/2020 - "Patients with type 1 von Willebrand disease (VWD) undergo a desmopressin (DDAVP) responsiveness challenge at diagnosis to assess whether DDAVP reverses their coagulation deficits. "
08/01/2020 - "Desmopressin responsiveness by age in type 1 von Willebrand disease."
06/01/2019 - "Quantitative analysis of desmopressin (DDAVP) response in adult patients with type 1 von Willebrand disease."
03/01/2016 - "The utility of VWF multimer analysis in response to the desmopressin administration for the diagnosis of severe type 1 von Willebrand disease."

12/01/2022 - "A multimer analysis of vWF showed a normal multimer pattern and he was diagnosed with von Willebrand disease type 1. As the bleeding tendency was mild, replacement of factor VIII was not performed. "
01/01/2021 - "ABSTRACT: Background Increasing levels of von Willebrand factor (VWF) and factor VIII (FVIII:C) was associated with age in type 1 von Willebrand disease (VWD). "
11/01/2020 - "Peripartum replacement of factor VIII and von Willebrand factor is not usually required in type 1 von Willebrand disease, as the levels of endogenous factors tend to increase to within the normal range as a physiological change of pregnancy. "
08/01/2013 - "Safety and pharmacokinetics (PK) of recombinant von Willebrand factor (rVWF) combined at a fixed ratio with recombinant factor VIII (rFVIII) were investigated in 32 subjects with type 3 or severe type 1 von Willebrand disease (VWD) in a prospective phase 1, multicenter, randomized clinical trial. "
02/01/1996 - "The defective interaction between von Willebrand factor and factor VIII in a patient with type 1 von Willebrand disease is caused by substitution of Arg19 and His54 in mature von Willebrand factor."

12/01/2018 - "Quantitative trait loci influencing VWF glycosylation, secretion and clearance have been associated with plasma VWF antigen levels in normal individuals, and may contribute to quantitative VWF abnormalities in patients with a thrombotic tendency or type 1 von Willebrand disease (VWD). "
01/01/2003 - "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). "
04/01/2014 - "Based on both reduced level of vWF antigen (vWF:Ag, range 14-56%) and ristocetin cofactor activity (vWF:RCo, range 12-53%), with a vWF:RCo/Ag ratio >0.7, the diagnosis of type 1 von Willebrand disease was made in all six cases. "
09/01/2010 - "Validation of a rapid test (VWF-LIA) for the quantitative determination of von Willebrand factor antigen in type 1 von Willebrand disease diagnosis within the European multicenter study MCMDM-1VWD."
10/01/1998 - "Two members of a family previously classified as type 1 von Willebrand disease (VWD), showed a quantitative defect in von Willebrand factor (VWF) antigen and ristocetin cofactor activity and an abnormal capacity of VWF to bind FVIII. "

10/01/2023 - "Efficacy of adjusted weight-based dosing of desmopressin (1-deamino-8-d-arginine vasopressin in type 1 von Willebrand disease."
07/01/2004 - "Desmopressin [1-deamino-8-d-arginine vasopressin (DDAVP)] has been successfully used in the treatment of type 1 von Willebrand disease (VWD) and mild haemophilia A (MHA). "

10/01/2010 - "Owing to its ability to raise plasma levels of factor VIII and von Willebrand factor levels, the synthetic vasopressin analogue desmopressin has become the mainstay of treatment for type 1 von Willebrand disease and mild hemophilia A. "

09/01/2014 - "Apart from representing an increased thrombotic risk, additional considerations entail the potential need (1) to utilize age-adjusted normal ranges (e.g., for D-dimer), (2) to consider the consequence on previous diagnoses (e.g., "mild type 1" von Willebrand disease [VWD], where VWF test results may "normalize" with aging), and (3) to consider the effect of these changes of risk factors on the (perceived) therapeutic efficacy of antithrombotic medications such as aspirin. "

09/01/2014 - "Apart from representing an increased thrombotic risk, additional considerations entail the potential need (1) to utilize age-adjusted normal ranges (e.g., for D-dimer), (2) to consider the consequence on previous diagnoses (e.g., "mild type 1" von Willebrand disease [VWD], where VWF test results may "normalize" with aging), and (3) to consider the effect of these changes of risk factors on the (perceived) therapeutic efficacy of antithrombotic medications such as aspirin. "

09/01/1998 - "Measurement of the von Willebrand factor (vWF) propeptide, also known as von Willebrand antigen II, has been suggested to be helpful in the discrimination of congenital von Willebrand disease type I from type 2 and in assessing the extent of activation of the endothelium. "

03/21/2013 - "We analyzed the contribution of VWFpp and ratios of VWFpp/VWF:Ag and FVIII:C/VWF:Ag in the pathophysiological characterization of type 1 von Willebrand disease (VWD) in the Molecular and Clinical Markers for the Diagnosis and Management of Type 1 VWD (MCMDM-1VWD) study. "
01/01/2007 - "Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD)."
04/01/2006 - "Linkage analysis in families diagnosed with type 1 von Willebrand disease in the European study, molecular and clinical markers for the diagnosis and management of type 1 VWD."
10/01/2018 - "To administer the condensed molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease VWD (MCMDM-1 vWD) questionnaire to the Omani type 1 vWD patients and correlate it with the laboratory parameters. "
01/01/2020 - "Using multiplex ligation-dependent probe amplification, this study determined the extent to which CNV contributed to VWD in the Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease cohort, highlighting in-frame deletions of exons 3, 4-5, 32-34, and 33-34. "

01/01/2020 - "Using multiplex ligation-dependent probe amplification, this study determined the extent to which CNV contributed to VWD in the Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease cohort, highlighting in-frame deletions of exons 3, 4-5, 32-34, and 33-34. "

09/01/2009 - "In brief, both therapies tended to normalize VWF test parameters and closure times in individuals with type 1 von Willebrand disease, with the level of correction in closure times related to the level of normalization of VWF, particularly the VWF:CB. "
10/01/2020 - "A novel microchip flow chamber system, the total thrombus-formation analysis system (T-TAS), is a point-of-care system to evaluate the thrombus-formation process and useful for monitoring platelet thrombus-formation capacity in patients receiving antiplatelet therapy and the diagnosis and evaluation of the clinical severity of von Willebrand disease type 1. However, little is known about the association between AVWS and platelet thrombus-formation capacity evaluated by T-TAS in patients with CF-LVAD. "
01/01/1992 - "Valproate therapy induces von Willebrand disease type I."

07/01/2007 - "Epidural analgesia for parturients with type 1 von Willebrand disease."
12/01/2003 - "A 33-year-old primipara with von Willebrand disease type I was admitted in labor at 37 weeks, requesting epidural analgesia. "

01/01/2009 - "Considerations for epidural anesthesia in a patient with type 1 von Willebrand disease."
01/01/2009 - "Epidural anesthesia, combined with general anesthesia, was performed in a patient with type 1 von Willebrand disease (VWD). "
12/01/2003 - "[Treatment with desmopressin before epidural anesthesia in a patient with type I von Willebrand disease]."

12/01/2015 - "We report the case of a 42-year-old woman, with type I von Willebrand disease, who underwent off-pump coronary artery bypass grafting. "