VON WILLEBRAND DISEASE
Described by Erik Adolf von Willebrand in 1926, it is a constitutional hemorrhagic disease, inherited in the autosomal mode. Considered initially as a thrombopathic disease, then as a prologation of the bleeding time associated to a defi cit in the factor VIII, today it is defi ned as an absence,...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Amaltea Medical Publishing House
2009-11-01
|
| Series: | Romanian Journal of Pediatrics |
| Subjects: | |
| Online Access: | https://revistemedicale.amaltea.ro/Romanian_Journal_of_PEDIATRICS/Revista_Romana_de_PEDIATRIE-2009-Nr.4/RJP_2009_4_Art-08.pdf |
| Summary: | Described by Erik Adolf von Willebrand in 1926, it is a constitutional hemorrhagic disease, inherited in the
autosomal mode. Considered initially as a thrombopathic disease, then as a prologation of the bleeding time
associated to a defi cit in the factor VIII, today it is defi ned as an absence, diminuation or a modifi cation of the
von Willebrand factor, a glycoprotein sintetized by the endotheklial cell and the megariocyte. The incidence
of the disease is around 2%, being the fi rst constitutionally hemorrhagic defi cit, in front of the hemophilias.
In this article we present: phisiopathologic mechanisms of the disease, clinical signs, biologic diagnosis,
classifi cation of von Willebrand disease, the differential diagnosis and treatment |
|---|---|
| ISSN: | 1454-0398 2069-6175 |