title: [History of congenital thrombocytic hemorrhagic dystrophy].
abstract: This historical review of the Bernard-Soulier syndrome relates: (1) the first description of the disease; (2) the main data of the research from 1948 to 1982 which led to the discovery of the molecular abnormality (lack or qualitative abnormality of the platelet glycoprotein Ib) responsible for the functional disorder (defective platelet adhesion to the vascular subendothelium) which nowadays defines the disorder.
authors.affiliation: Institut d'hématologie, Hôpital Saint-Louis, Paris, France.
authors.forename: J
authors.initials: J
authors.lastname: Bernard
mesh_terms: D001606:Bernard-Soulier Syndrome / Q000175:diagnosis / Q000235:genetics / Q000266:history*; D002675:Child, Preschool; D005260:Female; D049673:History, 20th Century; D006801:Humans; D008297:Male; D019038:Platelet Glycoprotein GPIb-IX Complex / Q000235:genetics
chemical_list: D019038:Platelet Glycoprotein GPIb-IX Complex
vernacular_title: Histoire de la dystrophie thrombocytaire hémorragipare (DTH) congénitale.