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Partial-Liver Transplantation To Treat Familial Amyloid Polyneuropathy: Follow-up of 11 Patients

Yo-ichi Takei, MD; Shu-ichi Ikeda, MD; Yasuhiko Hashikura, MD; Toshihiko Ikegami, MD; and Seiji Kawasaki, MD
[+] Article, Author, and Disclosure Information

From Shinshu University School of Medicine, Matsumoto, Japan.

Grant Support: In part by a research grant from the Intractable Disease Division, Ministry of Health and Welfare, Primary Amyloidosis Research Committee, Japan; a group research grant for the pathogenesis and therapy for hereditary neuropathy (10B-3); and a Grant-in-Aid for Scientific Research, Ministry of Education, Science and Culture, Japan (07457124).

Requests for Reprints: Shu-ichi Ikeda, MD, Department of Medicine, Neurology, Shinshu University School of Medicine, Matsumoto 390-8621, Japan; e-mail, ikedasi@hsp.md.shinshu-u.ac.jp.

Current Author Addresses: Drs. Takei and Ikeda: Department of Medicine, Neurology, Shinshu University School of Medicine, Matsumoto 390-8621, Japan.

Drs. Hashikura, Ikegami, and Kawasaki: Department of Surgery, Shinshu University School of Medicine, Matsumoto 390-8621, Japan.

Ann Intern Med. 1999;131(8):592-595. doi:10.7326/0003-4819-131-8-199910190-00008
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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.


Grahic Jump Location
Myelinated fiber lesions in sural nerve biopsy specimens. A.22222B.2C.2

Patient 2. Density of myelinated fibers, 5400/mm of endometrial area in sural nerve. Density of small myelinated fibers (<6 µm), 2439/mm (control, 4030 ± 457/mm ); density of large myelinated fibers (≥ 6 µm), 2961/mm (control, 3370 ± 163/mm ). Small myelinated fibers are more selectively depleted. Patient 6. Density of myelinated fibers, 1958/mm . Both small and large myelinated fibers are moderately affected. Patient 10. Density of myelinated fibers, 158/mm . There is a near-total loss of myelinated fibers. Epon-embedded sections stained with toluidine blue (original magnification, × 90). Bars = 100 µm.

Grahic Jump Location




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Summary for Patients

Transplant of Partial Livers to Treat a Rare Nerve Disorder

The summary below is from the full report titled “Partial-Liver Transplantation To Treat Familial Amyloid Polyneuropathy: Follow-up of 11 Patients.” It is in the 19 October 1999 issue of Annals of Internal Medicine (volume 131, pages 592-595). The authors are Y. Takei, S. Ikeda, Y. Hashikura, T. Ikegami, and S. Kawasaki.


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