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 Medjournal_title
Annals of internal medicineauthors
Takei Y,Ikeda S,Hashikura Y,Ikegami T,Kawasaki Sdoi
10.7326/0003-4819-131-8-199910190-00008subject
Has Abstractpub_date
1999-10-19 00:00:00pages
592-5issue
8eissn
0003-4819issn
1539-3704pii
199910190-00008journal_volume
131pub_type
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