title: Partial-liver transplantation to treat familial amyloid polyneuropathy: follow-up of 11 patients.
abstract: BACKGROUND
  Recently, liver transplantation has been used to treat patients with familial amyloid polyneuropathy (FAP).
  
  
  OBJECTIVE
  To describe the clinical course of patients with FAP who received partial-liver transplantation from living donors.
  
  
  METHODS
  Case series.
  
  
  METHODS
  University hospital in Matsumoto, Japan.
  
  
  METHODS
  11 patients with FAP who underwent partial-liver transplantation. The transthyretin gene abnormality in all 11 patients was the substitution of methionine for valine at position 30.
  
  
  METHODS
  Partial liver transplantation from living donors.
  
  
  METHODS
  Preoperative and follow-up (3 to 64 months) clinical data, including routine laboratory data, nerve conduction velocity tests, and sural nerve histology.
  
  
  RESULTS
  All 7 patients who had severe gastrointestinal autonomic disorders or polyneuropathy localized to the lower limbs for less than 4 years showed improvement. Three of 4 patients with polyneuropathy involving both the upper and lower limbs had adverse outcomes, including two deaths. The preoperative duration of their illness was more than 6 years. These 3 patients also had marked decreases in creatinine clearance and nerve conduction velocities and severe loss of myelinated fibers in sural nerves.
  
  
  CONCLUSIONS
  Preoperative clinical severity and duration of illness are associated with outcomes after liver transplantation for FAP.
authors.affiliation: Department of Medicine, Neurology, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Takei
authors.forename: S
authors.initials: S
authors.lastname: Ikeda
authors.forename: Y
authors.initials: Y
authors.lastname: Hashikura
authors.forename: T
authors.initials: T
authors.lastname: Ikegami
authors.forename: S
authors.initials: S
authors.lastname: Kawasaki
mesh_terms: D000328:Adult; D017772:Amyloid Neuropathies / Q000097:blood / Q000150:complications / Q000601:surgery*; D001806:Blood Urea Nitrogen; D003404:Creatinine / Q000097:blood; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction; D011183:Postoperative Complications; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D003404:Creatinine