Kostopanagiotou Georgia, Siafaka Ioanna, Sikiotis Constantinos, Smyrniotis Vassilios
Department of Anesthesiology, School of Medicine, University of Athens, Athens, Greece.
J Clin Anesth. 2004 Sep;16(6):458-60. doi: 10.1016/j.jclinane.2003.10.007.
We describe a patient with Bernard-Soulier syndrome who underwent emergency laparotomy for intraabdominal bleeding. Presentation of this recent case highlights some of the anesthetic and perioperative implications of the disease. Briefly, perioperative and anesthetic management of patients with Bernard-Soulier syndrome should include platelet transfusions and choice of appropriate anesthetic drugs and techniques. The potential benefit of desmopressin and corticosteroids should be considered. Thrombelastography, if available, is also useful.
我们描述了一名患有伯纳德-索利尔综合征的患者，该患者因腹腔内出血接受了急诊剖腹手术。这一近期病例的呈现突出了该疾病的一些麻醉及围手术期影响。简而言之，伯纳德-索利尔综合征患者的围手术期和麻醉管理应包括输注血小板以及选择合适的麻醉药物和技术。应考虑去氨加压素和皮质类固醇的潜在益处。如有条件，血栓弹力图检查也很有用。
