title: Inherited platelet disorders and oral health.
abstract: Platelets play a key role in thrombosis and hemostasis. Accumulation of platelets at the site of vascular injury is the first step in the formation of hemostatic plugs, which play a pivotal role in preventing blood loss after injury. Platelet adhesion at sites of injury results in spreading, secretion, recruitment of additional platelets, and formation of platelet aggregates. Inherited platelet disorders are rare causes of bleeding syndromes, ranging from mild bruising to severe hemorrhage. The defects can reflect deficiency or dysfunction of platelet surface glycoproteins, granule contents, cytoskeletal proteins, platelet pro-coagulant function, and signaling pathways. For instance, Bernard-Soulier syndrome and Glanzmann thrombasthenia are attributed to deficiencies of glycoprotein Ib/IX/V and GPIIb/IIIa, respectively, and are rare but severe platelet disorders. Inherited defects that impair platelet secretion and/or signal transduction are among the most common forms of mild platelet disorders and include gray platelet syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome. When necessary, desmopressin, antifibrinolytic agents, and transfusion of platelets remain the most common treatment of inherited platelet disorders. Alternative therapies such as recombinant activated factor VII are also available for a limited number of situations. In this review, we will discuss the management of patients with inherited platelet disorders in various clinical situations related to dental cares, including surgical intervention.
authors.affiliation: INSERM, U1048, Université Toulouse 3, I2MC, Equipe 11, CHU-Rangueil, Toulouse, France.
authors.forename: Marie-Cécile
authors.initials: MC
authors.lastname: Valera
authors.forename: Philippe
authors.initials: P
authors.lastname: Kemoun
authors.forename: Sarah
authors.initials: S
authors.lastname: Cousty
authors.forename: Pierre
authors.initials: P
authors.lastname: Sie
authors.forename: Bernard
authors.initials: B
authors.lastname: Payrastre
mesh_terms: D001606:Bernard-Soulier Syndrome / Q000235:genetics; D001791:Blood Platelet Disorders / Q000235:genetics*; D001792:Blood Platelets / Q000502:physiology; D017742:Dental Care for Chronically Ill; D006487:Hemostasis / Q000502:physiology; D006490:Hemostatics / Q000145:classification / Q000627:therapeutic use; D006801:Humans; D006472:Oral Hemorrhage / Q000517:prevention & control; D013915:Thrombasthenia / Q000235:genetics
chemical_list: D006490:Hemostatics