title: [Clinical and biological studies in the Bernard-Soulier syndrome (author's transl)].
abstract: Bernard-Soulier syndrome is a rare constitutional thrombopathy, the main clinical feature is a bleeding tendency which is variable. The long bleeding time, in spite of a normal or little decreased platelet count, has been recently explained: the giant platelets have a defective adhesion to subendothelium. This abnormal adhesion could be related with an abnormal pattern of platelet membrane glycoproteins. Interactions between vessel wall, von Willebrand's factor and platelets are discussed.
mesh_terms: D000244:Adenosine Diphosphate; D001779:Blood Coagulation Factors / Q000032:analysis; D001792:Blood Platelets / Q000378:metabolism; D002462:Cell Membrane / Q000378:metabolism; D002470:Cell Survival; D003094:Collagen; D005169:Factor VIII / Q000032:analysis; D005172:Factor XI / Q000032:analysis; D006023:Glycoproteins / Q000097:blood / Q000502:physiology; D006801:Humans; D010973:Platelet Adhesiveness; D010974:Platelet Aggregation; D011696:Purpura, Thrombocytopenic / Q000235:genetics*; D012310:Ristocetin / Q000494:pharmacology; D012701:Serotonin / Q000378:metabolism; D012794:Sialic Acids / Q000097:blood; D013577:Syndrome; D013925:Thromboplastin / Q000032:analysis; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D001779:Blood Coagulation Factors; D006023:Glycoproteins; D012794:Sialic Acids; D014841:von Willebrand Factor; D012310:Ristocetin; D012701:Serotonin; D000244:Adenosine Diphosphate; D005169:Factor VIII; D003094:Collagen; D005172:Factor XI; D013925:Thromboplastin
vernacular_title: Données cliniques et biologiques sur la dystrophie thrombocytaire hémorragipare