ACQUIRED AMYLOID NEUROPATHY IN A PORTUGUESE PATIENT AFTER DOMINO LIVER TRANSPLANTATION

作者：Conceicao Isabel^*; Evangelista Teresinha; Castro Jose; Pereira Pedro; Silvestre Ana; Coutinho Conceicao A; de Carvalho Mamede

来源：Muscle & Nerve, 2010, 42(5): 836-839.

DOI：10.1002/mus.21806

• domino liver transplantation
• familial amyloid polyneuropathy
• nerve conduction
• sympathetic skin response
• TTR

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 FAR 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. Muscle Nerve 42: 836-839, 2010