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- Title
Partial-liver transplantation to treat familial amyloid polyneuropathy: follow-up of 11 patients.
- Authors
Takei, Yo-ichi; Ikeda, Shu-ichi; Takei, Y; Ikeda, S; Hashikura, Y; Ikegami, T; Kawasaki, S
- Abstract
<bold>Background: </bold>Recently, liver transplantation has been used to treat patients with familial amyloid polyneuropathy (FAP).<bold>Objective: </bold>To describe the clinical course of patients with FAP who received partial-liver transplantation from living donors.<bold>Design: </bold>Case series.<bold>Setting: </bold>University hospital in Matsumoto, Japan.<bold>Patients: </bold>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.<bold>Intervention: </bold>Partial liver transplantation from living donors.<bold>Measurements: </bold>Preoperative and follow-up (3 to 64 months) clinical data, including routine laboratory data, nerve conduction velocity tests, and sural nerve histology.<bold>Results: </bold>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.<bold>Conclusion: </bold>Preoperative clinical severity and duration of illness are associated with outcomes after liver transplantation for FAP.
- Subjects
JAPAN; MATSUMOTO-shi (Japan); LIVER transplantation; POLYNEUROPATHIES
- Publication
Annals of Internal Medicine, 1999, Vol 131, Issue 8, p592
- ISSN
0003-4819
- Publication type
journal article
- DOI
10.7326/0003-4819-131-8-199910190-00008