title: Iatrogenic amyloid polyneuropathy after domino liver transplantation.
abstract: Liver transplantation has been used in treatment of transthyretin amyloidosis, and some patients undergo domino liver transplantation (DLT) with explanted liver being transplanted to another patient with liver failure as the liver is otherwise usually functionally normal. Until end of 2015, there were 1154 DLT performed worldwide. DLT for transthyretin amyloidosis is associated with the risk of developingde novosystemic amyloidosis and amyloid neuropathy, and the risk may be greater with some non-Val30Met mutations.De novoamyloid neuropathy has been described in up to 23% of transplant recipients. Neuropathy may be preceded by asymptomatic amyloid deposition in various tissues and symptoms of neuropathy started after a median of 7 years following DLT (5.7 ± 3.2 years; range 2 mo to 10 years). Typical initial symptoms include neuropathic pain and sensory loss, while dysautonomia usually starts later. Progression of neuropathy may necessitate liver re-transplantation, and subsequent improvement of neuropathy has been reported in some patients. Explant allograft recipients need close monitoring for signs of systemic amyloidosis, neuropathy and dysautonomia as progressive symptoms may require re-transplantation.
authors.affiliation: Diana Mnatsakanova, Saša A Živković, Department of Neurology, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, United States.
authors.forename: Diana
authors.initials: D
authors.lastname: Mnatsakanova
authors.affiliation: Diana Mnatsakanova, Saša A Živković, Department of Neurology, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, United States.
authors.forename: Saša A
authors.initials: SA
authors.lastname: Živković
keywords: Acquired amyloid neuropathy; Domino liver transplantation; Familial amyloid neuropathy; Systemic amyloidosis; Transthyretin