Sarah M Baker1, Ashley Kiefer2, Dominic S Carollo3,4, Rajasekharan P Warrier4,5. 1. Department of Microbiology and Immunology, Tulane University School of Medicine, New Orleans, LA. 2. Le Bonheur Children's Hospital, University of Tennessee, Memphis, TN. 3. Department of Anesthesiology, Ochsner Clinic Foundation, New Orleans, LA. 4. The University of Queensland School of Medicine, Ochsner Clinical School, New Orleans, LA. 5. Department of Pediatrics, Ochsner Clinic Foundation, New Orleans, LA.
Abstract
BACKGROUND: Prekallikrein deficiency is an extremely rare disorder in which functional prekallikrein in the plasma is reduced or absent. CASE REPORT: We present the case of a 15-year-old male with prolonged activated clotting time incidentally noted preoperatively prior to repair of an atrial septal defect. The patient was subsequently found to have prekallikrein (Fletcher factor) deficiency. He successfully underwent open cardiac surgical repair after fresh frozen plasma was administered at a dose of 15 mL/kg 1 hour prior to the start of the procedure. CONCLUSION: History and routine preoperative evaluations of complete blood count, partial thromboplastin time, prothrombin time, and platelet function analysis failed to detect any abnormalities, but a prolongation of activated clotting time identified by the anesthesiologist led to specialized testing and a diagnosis of Fletcher factor deficiency. The patient tolerated the open-heart surgery well without any significant hematologic intervention or complications.
BACKGROUND: Prekallikrein deficiency is an extremely rare disorder in which functional prekallikrein in the plasma is reduced or absent. CASE REPORT: We present the case of a 15-year-old male with prolonged activated clotting time incidentally noted preoperatively prior to repair of an atrial septal defect. The patient was subsequently found to have prekallikrein (Fletcher factor) deficiency. He successfully underwent open cardiac surgical repair after fresh frozen plasma was administered at a dose of 15 mL/kg 1 hour prior to the start of the procedure. CONCLUSION: History and routine preoperative evaluations of complete blood count, partial thromboplastin time, prothrombin time, and platelet function analysis failed to detect any abnormalities, but a prolongation of activated clotting time identified by the anesthesiologist led to specialized testing and a diagnosis of Fletcher factor deficiency. The patient tolerated the open-heart surgery well without any significant hematologic intervention or complications.
Authors: B Lämmle; W A Wuillemin; I Huber; M Krauskopf; C Zürcher; R Pflugshaupt; M Furlan Journal: Thromb Haemost Date: 1991-02-12 Impact factor: 5.249