title: Ten years of experience with liver transplantation for familial amyloid polyneuropathy in Japan: outcomes of living donor liver transplantations.
abstract: OBJECTIVE
  We summarize 10 years of experience with liver transplantation for FAP patients in Japan and review the current opinions regarding this treatment for FAP.
  
  
  METHODS
  All basic report data on patients at the time of transplantation were registered with the Japanese Liver Transplantation Society (JLTS). Based on the JLST report data, more detailed information on FAP patients was requested from each center.
  
  
  RESULTS
  Living donor liver transplantation (LDLT) for FAP patients was first performed in Japan in 1993. LDLT has since been performed in 41 FAP patients, including nine cases of temporary auxiliary partial orthotopic liver transplantation (APOLT). Orthotopic liver transplantation (OLT) from cadaveric donors for FAP patients began in 1999, but only one FAP patient has subsequently undergone this procedure. Of these total of 43 FAP patients, 36 are currently alive: the one-year survival rate of patients after transplantation was 93%, and the five-year survival rate of these cases was 77%. Preoperative clinical severity and the nutritional status of patients are correlated with their outcome after liver transplantation. Domino (sequential) liver transplantation has been carried out in 20 domino recipients with end-stage liver diseases. Of the 20 domino recipients, 12 are currently alive.
  
  
  CONCLUSIONS
  For FAP patients, these outcomes after the operation were very similar to those of OLT from cadaveric donors reported in other countries. Therefore, we concluded that for the treatment of FAP, LDLT from a living donor is equally effective as OLT from a cadaveric donor.
authors.affiliation: Third Department of Medicine, Shinshu University School of Medicine, Matsumoto.
authors.forename: Yo-Ichi
authors.initials: Y
authors.lastname: Takei
authors.forename: Shu-Ichi
authors.initials: S
authors.lastname: Ikeda
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Ikegami
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Hashikura
authors.forename: Shin-Ichi
authors.initials: S
authors.lastname: Miyagawa
authors.forename: Yukio
authors.initials: Y
authors.lastname: Ando
mesh_terms: D000328:Adult; D028227:Amyloid Neuropathies, Familial / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007564:Japan / Q000453:epidemiology; D016031:Liver Transplantation / Q000379:methods / Q000401:mortality; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D016896:Treatment Outcome