[Anesthetic management of a patient with hemophilia B during scoliosis surgery].
Posterior spinal fusion for scoliosis was planned in a 14-year-old male patient with hemophilia B. Preoperative examination showed factor IX activity of 8.4% with no inhibitor development. A perioperative dosage schedule was prepared after examining the pharmacokinetics of recombinant coagulation factor IX in order to maintain levels of perioperative factor IX activity at < or = 80% for the first 6 days (days 0-6), and > or = 40% for days 7-14 postoperatively. The dose of recombinant coagulation factor IX was adjusted to maintain factor IX activity above 80%, while measuring coagulation activity every hour during the surgery. The patient showed a favorable course without hemorrhagic tendency. We could safely manage anesthesia without requiring allogeneic blood transfusion.
Department ofAnesthesiology, Kobe Children's Hospital, Kobe 654-0081.
Shohei
S
Makino
Yuki
Y
Nomura
Shizuko
S
Kabara
Saeko
S
Takatsuji
Tetsuro
T
Kagawa
D000293:Adolescent; D000768:Anesthesia, General / Q000379:methods*; D005164:Factor IX / Q000008:administration & dosage; D002836:Hemophilia B / Q000150:complications*; D006801:Humans; D008297:Male; D011994:Recombinant Proteins / Q000008:administration & dosage; D012600:Scoliosis / Q000601:surgery*; D013123:Spinal Fusion
D011994:Recombinant Proteins; D005164:Factor IX