title: Perioperative management of tonsilloadenoidectomy and circumcision of a patient with Bernard-Soulier syndrome: case report.
abstract: Bernard-Soulier syndrome is an autosomal recessive coagulopathy characterized by thrombocytopenia, prolonged bleeding time and large platelets. Because of the rarity of this disease, standard perioperative treatment protocols have not been developed and there are not enough available data for the management of surgical procedures. In this case, we successfully performed adenoidectomy and tonsillectomy and circumcision surgery concurrently under the preventive and intermittent transfusion of platelets. During hospitalization, no intraoperative or postoperative bleeding complications occurred.
authors: aDepartment of Pediatric Hematology, Medical Faculty bDepartment of Pediatrics cDepartment of Otolaryngology - Head and Neck Surgery dDepartment of Pediatric Surgery, Pamukkale University, Denizli, Turkey.
authors: Yasemin I
authors: YI
authors: Balci
authors: Ersin
authors: E
authors: Gözkeser
authors: Aziz
authors: A
authors: Polat
authors: Merve
authors: M
authors: Gürses
authors: Cüneyt O
authors: CO
authors: Kara
authors: Özkan
authors: Ö
authors: Herek
mesh_terms: D000233:Adenoidectomy; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D001606:Bernard-Soulier Syndrome / Q000097:blood / Q000601:surgery / Q000628:therapy*; D002648:Child; D002944:Circumcision, Male; D006801:Humans; D007430:Intraoperative Care; D008297:Male; D010976:Platelet Count; D017713:Platelet Transfusion; D014068:Tonsillectomy; D014148:Tranexamic Acid / Q000627:therapeutic use
chemical_list: D000933:Antifibrinolytic Agents; D014148:Tranexamic Acid