Perioperative management of Glanzmann's syndrome: how we did it!
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.
Depner
Christian
C
Department of Surgery, University Hospital Basel, Basel, Switzerland. ch.depner@web.de
Schmid
Roger
R
Kirchhoff
Philipp
P
Oertli
Daniel
D
D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D006552:Hernia, Inguinal / Q000601:surgery*; D006801:Humans; D008297:Male; D017713:Platelet Transfusion; D011994:Recombinant Proteins / Q000627:therapeutic use; D013915:Thrombasthenia / Q000188:drug therapy / Q000628:therapy*; D014148:Tranexamic Acid / Q000627:therapeutic use; D055815:Young Adult
D000933:Antifibrinolytic Agents; D011994:Recombinant Proteins; D014148:Tranexamic Acid; C103587:recombinant FVIIa; D015942:Factor VIIa