Diagnosis of von Willebrand disease in Argentina: a single institution experience

von Willebrand disease (VWD) is the most common autosomal bleeding disorder, mostlyinherited as dominant trait. VWD is due to deficiency/abnormality of von Willebrand factor (VWF). Thetrue prevalence of VWD is unknown, but estimated as 0.1% to 1% of the general population. The bleeding score (BS) wa...

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Bibliographic Details
Authors: Woods, Adriana Inés, Kempfer, Ana Catalina, Paiva Palomino, Juvenal Hernán, Blanco, Alicia Noemi, Sánchez Luceros, Analía Gabriela, Lazzari, María Ángela
Format: article
Status:Published version
Publication Date:2017
Country:Argentina
Institution:Consejo Nacional de Investigaciones Científicas y Técnicas
Repository:CONICET Digital (CONICET)
Language:English
OAI Identifier:oai:ri.conicet.gov.ar:11336/45265
Online Access:http://hdl.handle.net/11336/45265
Access Level:Open access
Keyword:DESMOPRESSIN
PHENOTYPE
GENOTYPE
VON WILLEBRAND DISEASE
https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
Description
Summary:von Willebrand disease (VWD) is the most common autosomal bleeding disorder, mostlyinherited as dominant trait. VWD is due to deficiency/abnormality of von Willebrand factor (VWF). Thetrue prevalence of VWD is unknown, but estimated as 0.1% to 1% of the general population. The bleeding score (BS) was used to evaluate the bleeding status of patients presenting atour institution. Laboratory analyses include: VWF:Ag, VWF:RCo, VWF:CB, multimeric pattern, VWFpropeptide, Desmopressin challenge test. Genotypic analysis comprises the study of specific exons of VWF,depending on the suspected variant.