Nomura K, Harioka T, Itoh T, Kitajima T, Uno K, Kagawa D, Sone T
Department of Anesthesia, Shimada Municipal Hospital.
Masui. 1993 Oct;42(10):1521-3.
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.
伯纳德-索利尔综合征（BSS）是一种遗传性出血性疾病，其特征为由于血小板聚集异常和巨大血小板导致出血时间延长。输注富含血小板的血浆是治疗该疾病患者出血发作的唯一可用方法。一名20岁患有伯纳德-索利尔综合征的女性计划在全身麻醉下进行下颌支矢状劈开截骨术。麻醉诱导采用芬太尼和地西泮，维持采用氧化亚氮、芬太尼和0.5%安氟醚。手术期间及术后未观察到出血倾向加重。我们认为，伯纳德-索利尔综合征患者应避免使用氟烷，因为它可能抑制血小板聚集并延长出血时间。
