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Bernard-Soulier syndrome
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. Additional recommended knowledge
See also
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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. |