title: [Anesthetic management of a patient with Bernard-Soulier syndrome].
abstract: Bernard-Soulier syndrome (BSS) is a hereditary hemorrhagic disease characterized by prolonged bleeding time due to abnormal platelet aggregation and giant platelets. Transfusion of platelet-rich plasma is the only treatment available for the hemorrhagic episodes in patients with this disease. A 20-year-old female with BBS was scheduled for sagittal osteotomy of the mandibular rami under general anesthesia. Anesthesia was induced with fentanyl and diazepam, and was maintained with nitrous oxide, fentanyl, and 0.5% enflurane. No exacerbation of the bleeding tendency was observed during or after the surgery. We consider that the use of halothane should be avoided in patients with BBS because it may inhibit the aggregation of platelets and prolong the bleeding time.
authors.affiliation: Department of Anesthesia, Shimada Municipal Hospital.
authors.forename: K
authors.initials: K
authors.lastname: Nomura
authors.forename: T
authors.initials: T
authors.lastname: Harioka
authors.forename: T
authors.initials: T
authors.lastname: Itoh
authors.forename: T
authors.initials: T
authors.lastname: Kitajima
authors.forename: K
authors.initials: K
authors.lastname: Uno
authors.forename: D
authors.initials: D
authors.lastname: Kagawa
authors.forename: T
authors.initials: T
authors.lastname: Sone
mesh_terms: D000328:Adult; D000768:Anesthesia, General; D001606:Bernard-Soulier Syndrome; D004737:Enflurane; D005260:Female; D005283:Fentanyl; D006801:Humans; D009609:Nitrous Oxide; D013514:Surgical Procedures, Operative
chemical_list: D004737:Enflurane; D009609:Nitrous Oxide; D005283:Fentanyl