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Title: Acquired amyloid neuropathy in a portuguese patient after domino liver transplantation
Author: Conceição, Isabel
Evangelista, Teresinha
Castro, José
Pereira, Pedro
Silvestre, Ana
Coutinho, Conceição A.
Carvalho, Mamede de
Keywords: Domino liver transplantation
Familial amyloid polyneuropathy
Nerve conduction
Sympathetic skin response
Issue Date: 2010
Publisher: Wiley-Blackwell
Citation: Muscle & Nerve, Volume 42, Issue 5, pages 836–838, November 2010
Abstract: Familial amyloid polyneuropathy (FAP) is a progressive neuropathy with autonomic dysfunction. Domino liver transplantation (DLT), in which the liver of an FAP patient is transplanted into another patient, is routinely applied to compensate for the shortage of available organs. We report a patient who developed a clinical picture of FAP 9 years after a DLT from an FAP donor. Electrophysiological, neuropathological, and autonomic tests were administered. The patient presented with typical clinical features of FAP. Electrophysiological investigation confirmed a moderate sensorimotor axonal and autonomic neuropathy. Sural nerve biopsy confirmed the presence of amyloid deposits in the endoneurium. Skin biopsy at the ankle showed reduced intraepidermal nerve fiber density. Our report shows that FAP can develop in a recipient of an FAP liver. This suggests that careful longitudinal study is required to evaluate the risk of FAP polyneuropathy in patients who undergo domino liver transplantation.
Description: Copyright © 2010 Wiley Periodicals, Inc.
The definitive version is available at
Peer review: yes
DOI: 10.1002/mus.21806
ISSN: 0148-639X
Appears in Collections:IMM - Artigos em Revistas Internacionais

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