Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
1977-12-29
pubmed:abstractText
The biological syndrome in von Willebrand's disease is presently defined by four parameters: bleeding time, factor VIII coagulant activity (VIII:C), Willebrand factor activity (VIIR:WF) and factor VIII related antigen (VIIR:AG). The study of 48 patients from 25 families confirms the heterogeneity of the von Willebrand's syndrome including both quantitative as well as qualitative defects. Classical von Willebrand's disease results from the quantitative decrease of a plasma protein named "Willebrand factor" which is the primary gene-product of an altosomal chromosome. Both severe and moderate forms can be observed. In very severe forms, the apparent lack of the VIIIR:AG as measured by an immunoradiometric assay suggest the possibility that some patients are homozygous. "Variants" of von Willebrand's disease have been recently described in some patients, based on the discrepancy between the observed subnormal levels of VIIIR:AG and low levels of VIIIR:WF. The qualitative abnormality of the factor VIII/Willebrand protein can be assessed by different methods such as double cross immuno-electrophoresis, or immunoradiometric assay.
pubmed:language
fre
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:author
pubmed:volume
18
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
371-82
pubmed:dateRevised
2009-11-19
pubmed:meshHeading
pubmed:year
1977
pubmed:articleTitle
[Von Willebrand's syndrome (author's transl)].
pubmed:publicationType
Journal Article, English Abstract