A 16-year-old adolescent boy with a history of severe hemophilia A is undergoing an elective inguinal hernia repair. Which of the following is the best option for preventing or treating a bleeding complication in the setting of this disease?
Hemophilia A is a coagulation disorder resulting from a deficiency or abnormality of factor VIII. Desmopressin (DDAVP) is a synthetic analogue of antidiuretic hormone that increases levels of factor VIII and von Willebrand factor. DDAVP can be used alone for mild hemophilia A, but is ineffective in severe forms of the disease. For severe hemophilia A, DDAVP is given in combination with an inhibitor of fibrinolysis such as eaminocaproic acid (AMICAR). Although FFP contains factor VIII, the levels are too low to prevent or control bleeding in hemophiliacs. Other agents used in treatment of hemophilia A include cryoprecipitate and specific factor VIII concentrates.
Fresh-frozen plasma
Combination of desmopressin and fresh-frozen plasma
Combination of e-aminocaproic acid and desmopressin
Factor IX concentrate
Preoperative assessment and monitoring in anaesthesia