title: Perioperative management of Glanzmann's syndrome: how we did it!
abstract: 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.
authors.affiliation: Department of Surgery, University Hospital Basel, Basel, Switzerland. ch.depner@web.de
authors.forename: Christian
authors.initials: C
authors.lastname: Depner
authors.forename: Roger
authors.initials: R
authors.lastname: Schmid
authors.forename: Philipp
authors.initials: P
authors.lastname: Kirchhoff
authors.forename: Daniel
authors.initials: D
authors.lastname: Oertli
mesh_terms: 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
chemical_list: D000933:Antifibrinolytic Agents; D011994:Recombinant Proteins; D014148:Tranexamic Acid; C103587:recombinant FVIIa; D015942:Factor VIIa