Partial-liver transplantation to treat familial amyloid polyneuropathy: follow-up of 11 patients.
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.
Takei
Y
Y
Department of Medicine, Neurology, Shinshu University School of Medicine, Matsumoto, Japan.
Ikeda
S
S
Hashikura
Y
Y
Ikegami
T
T
Kawasaki
S
S
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
D003404:Creatinine