title: Liver transplantation for a pediatric patient with hemophilia B.
abstract: Hemophilia exposes patients to greater risks of bleeding complications during the perioperative period. However, there are no current protocols for factor replacement during LT. We herein describe a case of pediatric living donor LDLT performed for a patient with hemophilia B using perioperative short-term factor replacement. A 4-yr-old female patient with an extrahepatic portosystemic shunt and asymptomatic hemophilia B (factor IX activity 18.7%) underwent an ABO-compatible LDLT using a left lobe graft. The bleeding volume was 2980 mL. Freeze-dried human blood coagulation factor IX concentrate (Novact M, Kaketsuken, Japan) was administered at the induction of anesthesia and at the end of LDLT by bolus infusion (80 U/kg) and was continued by bolus infusion (40 U/kg) on POD 1, 2, 3, and 4. On POD 1, 5, 8, and 12, the factor IX plasma levels were 34.5%, 64.9%, 43.5%, and 53.1%, respectively. The postoperative course was uneventful, and the patient is currently doing well at 2.5 yr after LDLT. Factor concentrate should be administered at the induction of anesthesia and at the end of LT by bolus infusion, and thereafter be continued for a few days after LT by bolus infusion.
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Tochigi, Japan. yuki371@jichi.ac.jp
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Sanada
authors.forename: Taizen
authors.initials: T
authors.lastname: Urahashi
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ihara
authors.forename: Taiichi
authors.initials: T
authors.lastname: Wakiya
authors.forename: Noriki
authors.initials: N
authors.lastname: Okada
authors.forename: Naoya
authors.initials: N
authors.lastname: Yamada
authors.forename: Koichi
authors.initials: K
authors.lastname: Mizuta
mesh_terms: D000017:ABO Blood-Group System; D002675:Child, Preschool; D005164:Factor IX / Q000627:therapeutic use; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000150:complications / Q000628:therapy; D006085:Graft Survival; D002836:Hemophilia B / Q000150:complications*; D006801:Humans; D022124:Hyperammonemia / Q000150:complications / Q000628:therapy; D017093:Liver Failure / Q000150:complications* / Q000628:therapy*; D008113:Liver Neoplasms / Q000150:complications / Q000628:therapy; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D016896:Treatment Outcome
chemical_list: D000017:ABO Blood-Group System; D005164:Factor IX