title: [Bernard-Soulier syndrome from the clinical description (1948) to the molecular era (1977) (author's transl)].
abstract: Bernard-Soulier syndrome is a constitutional thrombopathy with  an impaired platelet adhesion to the vessel wall. Since the first description in 1948 many works had been reported, and recently a molecular abnormality of the platelet membrane was shown. Interactions between specific membrane sites and platelet functions are now proposed.
mesh_terms: D001791:Blood Platelet Disorders / Q000151:congenital*; D001792:Blood Platelets / Q000503:physiopathology; D002462:Cell Membrane; D004586:Electrophoresis; D005165:Factor V / Q000378:metabolism; D005169:Factor VIII / Q000378:metabolism; D005172:Factor XI / Q000378:metabolism; D006023:Glycoproteins / Q000378:metabolism; D010974:Platelet Aggregation; D012794:Sialic Acids / Q000172:deficiency; D013577:Syndrome
chemical_list: D006023:Glycoproteins; D012794:Sialic Acids; D005165:Factor V; D005169:Factor VIII; D005172:Factor XI
vernacular_title: La dystrophie thrombocytaire hémorragipare congénitale syndrome de Bernard-Soulier de la description clinique (1948) a l'ère moléculaire (1977).