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Bernard-Soulier syndrome



Bernard-Soulier syndrome
Classification & external resources
ICD-10 D69.1
ICD-9 287.1
OMIM 231200
DiseasesDB 1356
eMedicine ped/230 
MeSH D001606

Bernard-Soulier syndrome (BSS, named after Jean Bernard and Jean Pierre Soulier) is a genetic deficiency of glycoprotein Ib (GpIb), the receptor for von Willebrand factor that is important in clot formation. It presents as a bleeding disorder due to the inability of platelets to bind and aggregate at sites of vascular endothelial injury. A good website for more information is www.B-SS.org.


See also

 
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Bernard-Soulier_syndrome". A list of authors is available in Wikipedia.
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