title: Living donor liver transplantation from an asymptomatic donor with mild coagulation factor IX deficiency: report of a case.
abstract: The use of donors with coagulation FIX deficiency is controversial, and there are no current protocols for peri-transplant management. We herein describe the first reported case of a pediatric LDLT from an asymptomatic donor with mild coagulation FIX deficiency. A 32-yr-old female was evaluated as a donor for her 12-month-old daughter with biliary atresia. The donor's pretransplant coagulation tests revealed asymptomatic mild coagulation FIX deficiency (FIX activity 60.8%). Freeze-dried human blood coagulation FIX concentrate was administered before the dissection of the liver and 12 h afterwards by bolus infusion (40 U/kg) and was continued on POD 1. The bleeding volume at LDLT was 590 mL. On POD 1, 3, 5, and 13, the coagulation FIX activity of the donor was 121.3%, 130.6%, 114.6%, and 50.2%, respectively. The donor's post-transplant course was uneventful, and the recipient is currently doing well at 18 months after LDLT. The FIX activity of the donor and recipient at nine months after LDLT was 39.2% and 58.0%, respectively. LDLT from donors with mild coagulation FIX deficiency could be performed effectively and safely using peri-transplant short-term coagulation FIX replacement and long-term monitoring of the plasma FIX level in the donor.
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Tochigi, Japan; Department of Surgery, Jichi Medical University, Shimotsuke City, Tochigi, Japan.
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Sanada
authors.forename: Hideki
authors.initials: H
authors.lastname: Sasanuma
authors.forename: Yasunaru
authors.initials: Y
authors.lastname: Sakuma
authors.forename: Kazue
authors.initials: K
authors.lastname: Morishima
authors.forename: Naoya
authors.initials: N
authors.lastname: Kasahara
authors.forename: Yuji
authors.initials: Y
authors.lastname: Kaneda
authors.forename: Atsushi
authors.initials: A
authors.lastname: Miki
authors.forename: Takehito
authors.initials: T
authors.lastname: Fujiwara
authors.forename: Atsushi
authors.initials: A
authors.lastname: Shimizu
authors.forename: Masanobu
authors.initials: M
authors.lastname: Hyodo
authors.forename: Yuta
authors.initials: Y
authors.lastname: Hirata
authors.forename: Naoya
authors.initials: N
authors.lastname: Yamada
authors.forename: Noriki
authors.initials: N
authors.lastname: Okada
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ihara
authors.forename: Taizen
authors.initials: T
authors.lastname: Urahashi
authors.forename: Seiji
authors.initials: S
authors.lastname: Madoiwa
authors.forename: Jun
authors.initials: J
authors.lastname: Mimuro
authors.forename: Koichi
authors.initials: K
authors.lastname: Mizuta
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Yasuda
mesh_terms: D000328:Adult; D058070:Asymptomatic Diseases; D001656:Biliary Atresia / Q000601:surgery*; D005260:Female; D002836:Hemophilia B; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors
keywords: coagulation factor IX activity; coagulation factor IX deficiency; freeze-dried human blood coagulation factor IX concentrate; living donor; living donor liver transplantation