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ICD9 = | ICDO = | OMIM = 231200 | MedlinePlus = | eMedicineSubj = ped | eMedicineTopic = 230 | MeshID = 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.
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