title: Efficacy of auxiliary partial orthotopic liver transplantation for cure of hemophilia in a canine hemophilia A model.
abstract: Metabolic liver disease can be cured by orthotopic liver transplantation. Some successful cases of whole or partial liver transplantation have been reported. Because liver function in these recipients is normal save for the production of the responsive metabolic factor, auxiliary partial orthotopic liver transplantation (APOLT) may produce a benefit. However, no experimental model of APOLT for metabolic liver diseases has been reported. We established a canine APOLT model to evaluate the clinical feasibility and efficacy of APOLT to cure hemophilia. The donor normal beagle dog was used to establish an APOLT model. A left lobe partial liver graft taken from the donor was orthotopically transplanted to the recipient after resection of the native left lobe preserving the native right lobe. Recipients showed no atrophy and comparable blood flow in both the graft and the native liver at the time of exploration after APOLT. Thus, APOLT was performed from a normal donor to a recipient with hemophilia A. In this recipient, blood factor VIII activity markedly increased after APOLT and was maintained for 7 weeks. No episode of bleeding was seen during the observation. In conclusion, a canine APOLT model was successfully established as evidenced by sustained production of factor VIII in a hemophilia recipient. These findings suggest the clinical feasibility and efficacy of APOLT for metabolic liver diseases.
authors.affiliation: Department of Surgery, Nara Medical University, Kashihara, Nara, Japan. saihoko@naramed-u.ac.jp
authors.forename: S
authors.initials: S
authors.lastname: Ko
authors.forename: I
authors.initials: I
authors.lastname: Tanaka
authors.forename: H
authors.initials: H
authors.lastname: Kanokogi
authors.forename: H
authors.initials: H
authors.lastname: Kanehiro
authors.forename: J
authors.initials: J
authors.lastname: Okayama
authors.forename: J
authors.initials: J
authors.lastname: Ori
authors.forename: M
authors.initials: M
authors.lastname: Shima
authors.forename: A
authors.initials: A
authors.lastname: Yoshioka
authors.forename: A
authors.initials: A
authors.lastname: Giles
authors.forename: Y
authors.initials: Y
authors.lastname: Nakajima
mesh_terms: D000818:Animals; D001663:Bilirubin / Q000097:blood; D015415:Biomarkers / Q000097:blood; D004195:Disease Models, Animal; D004285:Dogs; D005169:Factor VIII / Q000032:analysis*; D005240:Feasibility Studies; D006085:Graft Survival; D006467:Hemophilia A / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods* / Q000502:physiology; D008297:Male; D014184:Transplantation, Homologous
chemical_list: D015415:Biomarkers; D005169:Factor VIII; D001663:Bilirubin