• Login
    View Item 
    •   KovsieScholar Home
    • Health Sciences
    • Haematology and Cell Biology
    • Research Articles (Haematology and Cell Biology)
    • View Item
    •   KovsieScholar Home
    • Health Sciences
    • Haematology and Cell Biology
    • Research Articles (Haematology and Cell Biology)
    • View Item
    JavaScript is disabled for your browser. Some features of this site may not work without it.

    Laboratory diagnosis and management of von Willebrand disease in South Africa

    Thumbnail
    View/Open
    Meiring et al.pdf (196.4Kb)
    Date
    2011
    Author
    Meiring, Muriel
    Coetzee, Marius
    Kelderman, Mareli
    Badenhorst, Philip
    Metadata
    Show full item record
    Abstract
    Patients with von Willebrand disease (VWD) in South Africa are cared for in 17 Hemophilia Treatment Centers. The exact prevalence of the disease is uncertain, but 539 patients are annotated in registries. VWD patients are mostly diagnosed in the five largest academic centers, and the classification of the subtypes is performed by one of these, the VWD testing facility. An algorithm is used for the diagnosis of VWD. The distribution of subtypes diagnosed by the VWD reference center is 38%, 58%, and 4% for type 1, 2, and 3, respectively, and ~15% of plasma samples received are rejected due to poor storage and transport conditions. A novel single nucleotide polymorphism has been found in an African patient with type 2B VWD. From the type 1 VWDpatients who were diagnosed by the VWD testing facility, 45% seem to have an increased VWF clearance phenotype with a propeptide-to-antigen ratio of 1.9±0.3. VWD patients are treated with desmopressin, factor (F)VIII/VWF concentrate (Haemosolvate FVIII; National Bioproducts Institute, Durban, South Africa), and tranexamic acid. Haemosolvate FVIII contains a VWF antigen concentration of 167±27 IU/mL, a ristocetin cofactor activity of 100±29 IU/mL, a collagen binding activity of 99±29 IU/mL, normal VWF multimers, and a FVIII concentration of 50 IU/mL. Not all patients with VWD are currently classified, and many VWD patients in South Africa are probably undiagnosed.
    URI
    http://hdl.handle.net/11660/2377
    Collections
    • Research Articles (Haematology and Cell Biology)

    DSpace software copyright © 2002-2016  DuraSpace
    Contact Us | Send Feedback
     

     

    Browse

    All of KovsieScholarCommunities & CollectionsBy Issue DateAuthorsTitlesSubjectsThis CollectionBy Issue DateAuthorsTitlesSubjects

    My Account

    LoginRegister

    Statistics

    View Usage Statistics

    DSpace software copyright © 2002-2016  DuraSpace
    Contact Us | Send Feedback