Source:http://linkedlifedata.com/resource/pubmed/id/20008189
Switch to
| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:dateCreated |
2009-12-16
|
| pubmed:abstractText |
The wide clinical spectrum of von Willebrand disease (VWD), its complex pathophysiology and its classification into distinct quantitative (type 1 or type 3) and qualitative (type 2) types with further subtle distinctions have prevented most clinicians from establishing a straightforward approach to diagnosing and treating this inherited bleeding disorder. The results of studies involving large cohorts of patients with a wide range of bleeding manifestations and variable von Willebrand factor (VWF) reduction have recently become available. These data have allowed the proposal of minimal criteria for a clinically useful diagnosis and for differentiating patients with mild VWD from subjects with borderline or only slightly reduced VWF levels who will not benefit from a specific diagnosis. These criteria are based on measurement of VWF ristocetin cofactor (VWF:RCo), VWF antigen (VWF:Ag), factor VIII and a standardized bleeding score (BS). Demonstration of the inheritance of the disorder could help to classify patients for whom insufficient hemostatic challenges may produce a falsely reassuring BS (like in children). Using this approach, mild VWD appears to be mostly composed of type 1 cases. Complemented by the results of desmopressin trial infusion, these parameters form the basis for a clinically oriented classification of all forms of VWD and may be useful for selecting the best treatment according to the severity of the disease. Although few molecular data have revealed practical utility, there is no doubt that the clarification of the molecular pathophysiology of VWD has allowed the unification of this complex disorder into a simple conceptual framework. This framework underlies the proposed utilization of simple phenotypic markers for optimizing treatments in individual patients.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Deamino Arginine Vasopressin,
http://linkedlifedata.com/resource/pubmed/chemical/F8 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Factor VIII,
http://linkedlifedata.com/resource/pubmed/chemical/Von Willebrand antigen,
http://linkedlifedata.com/resource/pubmed/chemical/von Willebrand Factor
|
| pubmed:status |
MEDLINE
|
| pubmed:issn |
1520-4383
|
| pubmed:author | |
| pubmed:issnType |
Electronic
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
113-23
|
| pubmed:meshHeading |
pubmed-meshheading:20008189-Adult,
pubmed-meshheading:20008189-Child,
pubmed-meshheading:20008189-Deamino Arginine Vasopressin,
pubmed-meshheading:20008189-Disease Management,
pubmed-meshheading:20008189-Factor VIII,
pubmed-meshheading:20008189-Female,
pubmed-meshheading:20008189-Hemorrhage,
pubmed-meshheading:20008189-Humans,
pubmed-meshheading:20008189-Male,
pubmed-meshheading:20008189-Mutation,
pubmed-meshheading:20008189-Patient Selection,
pubmed-meshheading:20008189-Phenotype,
pubmed-meshheading:20008189-Postpartum Hemorrhage,
pubmed-meshheading:20008189-Pregnancy,
pubmed-meshheading:20008189-Pregnancy Complications, Hematologic,
pubmed-meshheading:20008189-Severity of Illness Index,
pubmed-meshheading:20008189-von Willebrand Diseases,
pubmed-meshheading:20008189-von Willebrand Factor
|
| pubmed:year |
2009
|
| pubmed:articleTitle |
Optimizing treatment of von Willebrand disease by using phenotypic and molecular data.
|
| pubmed:affiliation |
Department of Cell Therapy and Hematology, San Bortolo Hospital, Vicenza, Italy. rodeghiero@hemato.ven.it
|
| pubmed:publicationType |
Journal Article,
Review,
Research Support, Non-U.S. Gov't
|