Linked Life Data

12395174

Source:http://linkedlifedata.com/resource/pubmed/id/12395174

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
10
pubmed:dateCreated
2002-10-23
pubmed:abstractText
Von Willebrand disease (vWD) is the most widespread inherited bleeding disorder caused by quantitative or qualitative abnormalities of von Willebrand factor (vWF), an adhesive glycoprotein found in blood plasma and platelets and participating in primary and secondary/endothelium haemostasis as well. Although bleeding symptoms are often mild or moderate, patients with vWD represent a very heterogenous group with different phenotypes and a wide variability of the clinical pattern. In accordance with different defects of vWF, vWD is classified into various types and subtypes. This is illustrated by two case reports of patients with different types of vWD. Two main therapeutic options are available for the prevention and treatment of bleeding: desmopressin (DDAVP) and replacement therapy with plasma concentrates containing both factor VIII and vWF. DDAVP is the treatment of choice for most patients with type 1, representing about 80% of all patients with vWD. In patients with most types of type 2 and in all patients of type 3, DDAVP alone is ineffective or even contraindicated, and it is usually necessary to switch to plasma concentrates. Although treatment of vWD seems to be relatively simple in most cases, the exact diagnosis and phenotype characterization requires specialized or expert laboratory facilities. Furthermore, no reliable screening method for the diagnosis of vWD exists. Acquired vWD has similar clinical features and laboratory findings to the congenital forms and is mostly associated with lymphoproliferative or autoimmune disorders or neoplasia.
pubmed:language
ger
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
0003-2417
pubmed:author
pubmed:issnType
Print
pubmed:volume
51
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
825-34
pubmed:dateRevised
2009-11-19
pubmed:meshHeading
pubmed:year
2002
pubmed:articleTitle
[Perioperative therapy of von Willebrand disease. Demonstration of pathophysiology, clinical problems and therapy options using two case reports].
pubmed:affiliation
Klinik für Anaesthesiologie und Intensivmedizin, Universitätskliniken des Saarlandes, Homburg/Saar, Germany. aiskle@uniklinik-saarland.de
pubmed:publicationType
Journal Article, English Abstract, Review, Case Reports