Depner Christian, Schmid Roger, Kirchhoff Philipp, Oertli Daniel
Department of Surgery, University Hospital Basel, Basel, Switzerland.
Blood Coagul Fibrinolysis. 2010 Apr;21(3):283-4. doi: 10.1097/MBC.0b013e328330e683.
Thrombasthenia Glanzmann, named after the Swiss paediatrician Eduard Glanzmann (1887-1959), is a rare disease of platelet dysfunction. This disease is characterized by a deficiency or defect of the fibrinogen receptor (GPIIb-IIIa) on the platelet surface. The GPIIb-IIIa receptor has an essential function in the adhesion and aggregation of the platelets. The platelets of these patients cannot bind fibrinogen and aggregation does not occur. Patients have a severe lifelong risk of bleeding, especially during surgical procedures. These patients require a special perioperative regimen for an adequate function of the coagulation system to prevent intraoperative and postoperative bleeding. In this study, we present the perioperative management of a patient with thrombasthenia Glanzmann during elective inguinal hernia repair.
血小板无力症（Glanzmann血小板无力症），以瑞士儿科医生爱德华·格兰兹曼（1887 - 1959）命名，是一种罕见的血小板功能障碍疾病。这种疾病的特征是血小板表面纤维蛋白原受体（糖蛋白IIb - IIIa，GPIIb - IIIa）缺乏或缺陷。GPIIb - IIIa受体在血小板的黏附和聚集过程中具有重要作用。这些患者的血小板无法结合纤维蛋白原，因而不会发生聚集。患者终生面临严重的出血风险，尤其是在外科手术过程中。为使凝血系统充分发挥功能以预防术中及术后出血，这些患者需要特殊的围手术期治疗方案。在本研究中，我们介绍了一名Glanzmann血小板无力症患者在择期腹股沟疝修补术中的围手术期管理。
