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. DESIGN:Case series. SETTING:University hospital in Matsumoto, Japan. PATIENTS: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. INTERVENTION:Partial liver transplantation from living donors. MEASUREMENTS: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. CONCLUSION:Preoperative clinical severity and duration of illness are associated with outcomes after liver transplantation for FAP.

journal_name

Ann Intern Med

authors

Takei Y,Ikeda S,Hashikura Y,Ikegami T,Kawasaki S

doi

10.7326/0003-4819-131-8-199910190-00008

subject

Has Abstract

pub_date

1999-10-19 00:00:00

pages

592-5

issue

8

eissn

0003-4819

issn

1539-3704

pii

199910190-00008

journal_volume

131

pub_type

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