• Disease
  • Factor
  • Factor Viii
  • Eveline Pauline Mauser‐bunschoten
  • Eveline Pauline Mauser‐Bunschoten

    Eveline Pauline Mauser‐Bunschoten


    Prominent publications by Eveline Pauline Mauser‐Bunschoten

    KOL Index score: 16851

    BACKGROUND: von Willebrand factor (VWF) levels in healthy individuals are influenced by variations in genetic loci other than the VWF gene, whose contribution to VWF levels in patients with von Willebrand disease (VWD) is largely unknown.

    OBJECTIVES: To investigate the association between single-nucleotide polymorphisms (SNPs), VWF levels, and bleeding phenotype.

    PATIENTS/METHODS: In 364 type 1 VWD and 240 type 2 VWD patients from the nationwide cross-sectional 'Willebrand in The ...

    Also Ranks for: Willebrand Disease |  vwf levels |  1 vwd |  stxbp5 gene |  type 2
    KOL Index score: 12825

    Some comorbidities, such as hypertension, are associated with higher von Willebrand factor (VWF) levels in the general population. No studies have been conducted to assess this association in patients with von Willebrand disease (VWD). Therefore, we studied this association in patients with type 1 (n = 333) and type 2 (n = 203) VWD from the 'WiN" study. VWF antigen (VWF:Ag) was higher in type 1 VWD patients with hypertension [difference: 0·23 iu/ml, 95% confidence interval (CI): ...

    Also Ranks for: Willebrand Disease |  type 1 vwd |  vwf fviii |  win study |  factor viii
    KOL Index score: 11136

    The ratios between von Willebrand factor propeptide (VWFpp) or factor VIII activity (

    FVIII: C) and VWF antigen (VWF:Ag) reflect synthesis, secretion, and clearance of VWF. We aimed to define the pathophysiology of 658 patients with type 1, 2, or 3 von Willebrand disease (VWD) with VWF levels ≤30 U/dL from the Willebrand in The Netherlands (WiN) study using the VWFpp/VWF:Ag and

    FVIII: C/VWF:Ag ratios. We evaluated the use of VWFpp in the classification and diagnosis of VWD. On the basis ...

    Also Ranks for: Willebrand Factor Propeptide |  vwf vwfpp |  diagnosis vwd |  type 3 |  child child
    KOL Index score: 11135

    BACKGROUND: Initial clinical experience with recombinant factor VIIa (rVIIa) for treatment of haemophilia patients with inhibitors against factor VIII or IX has been obtained by administration of rVIIa by repeated intravenous bolus injections. However, continuous infusion of rVIIa may be a more appropriate administration method if prolonged treatment is indicated.

    METHODS: We have surveyed and analysed the initial experience with continuous infusion of rVIIa in the Netherlands and ...

    Also Ranks for: Continuous Infusion |  recombinant factor viia |  prothrombin time |  haemophilia patients |  factor viii
    KOL Index score: 10977

    Chronic hepatitis C is a major comorbidity in patients with haemophilia. Although the current state-of-the-art therapy consists of pegylated interferon (PegIFN) and ribavirin, there are no reports of the efficacy of this combination in the haemophilia population. The aim of this study was to assess the response and side-effects of PegIFN and ribavirin in patients with inherited bleeding disorders. Patients with chronic hepatitis C were treated with PegIFN alpha-2b (1.5 microg kg(-1) ...

    Also Ranks for: Chronic Hepatitis |  ribavirin patients |  pegylated interferon |  bleeding disorders |  combination therapy
    KOL Index score: 10409

    A second generation ELISA for combined detection of antibodies to three hepatitis C virus (HCV) recombinant proteins, i.e. C100, C33c and core, was compared with a first generation anti-HCV ELISA in which only antibodies to C100 are detected. The results of the ELISAs were evaluated in 225 haemophilia patients (panel A) and 44 patients with non-A, non-B (NANB) hepatitis (panel B). HCV infection was established by cDNA-polymerase chain reaction (PCR) (in panel B only) and by studying the ...

    Also Ranks for: Generation Elisa |  hepatitis virus |  hcv infection |  c33c c100 |  recombinant proteins
    KOL Index score: 10080

    BACKGROUND: Many patients with bleeding disorders have been infected with the hepatitis C virus (HCV), mainly with genotype 1. Antiviral treatment is only effective in 50% of these patients and is often accompanied by serious side effects. Consequently, careful selection of patients for treatment is warranted. Liver biopsies are generally not performed in these patients because of increased bleeding risk and high costs. We therefore assessed liver fibrosis and cirrhosis non-invasively ...

    Also Ranks for: Bleeding Disorders |  liver fibrosis |  chronic hepatitis |  antiviral treatment |  transient elastography
    KOL Index score: 10001

    Mild haemophilia A is a rare disease with a relatively mild phenotype. Treatment with factor VIII (FVIII) is indicated after trauma or for surgery only. FVIII infusion may result in the development of inhibiting antibodies against FVIII. This study describes the relation between age and other risk factors for inhibitor development in mild haemophilia. A retrospective cohort study was conducted among all patients with mild haemophilia (FVIII 0.05-0.40 IU mL(-1)) registered at the van ...

    Also Ranks for: Mild Haemophilia |  inhibitor development |  factor viii fviii |  peak treatment |  age patients
    KOL Index score: 9931

    BACKGROUND: Because the number of elderly von Willebrand disease (VWD) patients is increasing, the pathophysiology of aging in VWD has become increasingly relevant.

    OBJECTIVES: To assess age-related changes in von Willebrand factor (VWF) and factor VIII (FVIII) levels and to compare age-related differences in bleeding phenotype between elderly VWD patients and those < 65 years. We also studied co-morbidity in elderly patients.

    PATIENTS/METHODS: We included VWD patients with VWF levels ...

    Also Ranks for: Vwd Patients |  bleeding phenotype |  willebrand disease |  age type |  vwf fviii levels
    KOL Index score: 9921

    We performed a nation-wide cross-sectional study to evaluate determinants of bleeding symptoms in a large unselected cohort of adults with von Willebrand disease (VWD). VWD patients were included (n=664), based on lowest historically measured VWF:Ag and VWF:Act levels ≤30 U/dl. Menorrhagia (85%), cutaneous bleeding (77%), bleeding from minor wounds (77%) and oral-cavity bleeding (62%) occurred most frequently. Higher age was associated with a higher bleeding score (BS), determined ...

    Also Ranks for: Bleeding Phenotype |  willebrand disease |  vwf levels |  type 3 patients |  1 iu
    KOL Index score: 9861

    BACKGROUND: High von Willebrand factor (VWF) levels are an established risk factor for arterial thrombosis, including coronary heart disease and ischemic stroke. It has been hypothesized that von Willebrand disease (VWD) patients are protected against arterial thrombosis; however, this has never been confirmed in clinical studies.

    OBJECTIVES: To investigate the prevalence of arterial thrombosis in VWD patients relative to the general population.

    PATIENTS/METHODS: We included 635 adult ...

    Also Ranks for: Arterial Thrombosis |  willebrand disease |  ischemic stroke |  vwd patients |  aged prevalence
    KOL Index score: 9557

    BACKGROUND: The life expectancy of non-severe hemophilia A (HA) patients equals the life expectancy of the non-hemophilic population. However, data on the effect of inhibitor development on mortality and on hemophilia-related causes of death are scarce. The development of neutralizing factor VIII antibodies in non-severe HA patients may dramatically change their clinical outcome due to severe bleeding complications.

    OBJECTIVES: We assessed the association between the occurrence of ...

    Also Ranks for: Inhibitor Development |  severe hemophilia |  life expectancy |  mortality rates |  factor viii
    KOL Index score: 9487

    We have prospectively monitored treatment of haemophilia patients with inhibitors by recombinant factor VIIa (rFVIIa) administered by continuous infusion to obtain more insight in the underlying factors of the clinical efficacy of this administration method. At present, 43 treatment episodes of 14 different Dutch haemophilia inhibitor patients are included in the database. Analysis of the data showed a discrepancy between the efficacy of rFVIIa continuous infusion treatment of acute and ...

    Also Ranks for: Continuous Infusion |  haemophilia patients |  recombinant factor viia |  female hemophilia |  factor inhibitors


    Eveline Pauline Mauser‐Bunschoten: Influence Statistics

    Sample of concepts for which Eveline Pauline Mauser‐Bunschoten is among the top experts in the world.
    Concept World rank
    dose immune #1
    immune tolerance patients #1
    005219 iu #1
    40 ml1 #1
    haemophilia general #1
    june classic inhibitors #1
    11 haemophilia patients #1
    haemophilia patients agerelated #1
    hemophilic target joints #1
    766 questionnaires #1
    hemophilic target #1
    cyclophosphamide inhibitory antibodies #1
    development multitransfused #1
    contribution inhibitors #1
    disorders hepatitis #1
    profuse intraarticular #1
    060 iu range #1
    multitransfused patient #1
    hemophilia fewer #1
    noncarriers female carriers #1
    joint haemophilia #1
    infusion rviia #1
    domain specificity fviii #1
    european htcs hmb #1
    disappearance factor viii #1
    hcv hemophilia #1
    haemophilia addition #1
    highest inhibitor #1
    patient mild haemophilia #1
    haemophilia sports #1
    rviia prolonged treatment #1
    htcs hmb #1
    inherited cost #1
    inhibitor median age #1
    experience rviia #1
    43 treatment episodes #1
    heterozygous carriers hemophilia #1
    severe blush #1
    centres joint #1
    inherited cohort #1
    cavity acute #1
    haemophilia retrospective #1
    episodes feiba #1
    viia administered #1

    Key People For Factor Viii

    Top KOLs in the world
    Leon W Hoyer
    factor viii endothelial cells murine hemophilia
    Edward G D Tuddenham
    factor viii gene therapy monoclonal antibodies
    Pier Mannuccio Mannuccio Mannucci
    willebrand factor venous thromboembolism myocardial infarction
    Randal J Kaufman
    factor viii endoplasmic reticulum unfolded protein response
    Gordon A Vehar
    factor viii cytoplasmic domain plasminogen activator
    Pete Lollar
    factor viii immune response major determinant

    Eveline Pauline Mauser‐Bunschoten:Expert Impact

    Concepts for whichEveline Pauline Mauser‐Bunschotenhas direct influence:Factor viii,  Haemophilia patients,  Willebrand disease,  Severe hemophilia,  Severe haemophilia,  Hemophilia patients,  Chronic hepatitis,  Joint bleeds.

    Eveline Pauline Mauser‐Bunschoten:KOL impact

    Concepts related to the work of other authors for whichfor which Eveline Pauline Mauser‐Bunschoten has influence:Factor viii,  Severe haemophilia,  Willebrand disease,  Inhibitor development,  Bleeding disorders,  Patients hemophilia,  Immune tolerance.



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    University Medical Center Utrecht, Utrecht, Netherlands | Van Creveldkliniek, University Medical Center Utrecht, University Utrecht, Utrecht, The Netherlands | Van Creveldkliniek, Center for Benign Haematology, University Medical Center Utrecht, Utre