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Amyloid Neuropathy Following Domino Liver Transplantation Response to Diflunisal

Pierre R. Bourque, MD, FRCPC1; Sharmi Shafi, MD1; Gerard H. Jansen, MD, FRCPC2; Arleigh McCurdy, MD, FRCPC3; Jodi Warman Chardon, MD, FRCPC1,4,5
[+] Author Affiliations
1Division of Neurology, Department of Medicine, The Ottawa Hospital, Ottawa, Ontario, Canada
2Department of Pathology and Laboratory Medicine, The Ottawa Hospital, Ottawa, Ontario, Canada
3Department of Hematology, The Ottawa Hospital, Ottawa, Ontario, Canada
4Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
5Department of Genetics, Children’s Hospital of Eastern Ontario, Ottawa, Ontario, Canada
JAMA Neurol. 2016;73(4):477-478. doi:10.1001/jamaneurol.2015.4715.
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This case report describes possible stabilization of liver transplant–acquired amyloid neuropathy with diflunisal.

The transmission of systemic transthyretin amyloidosis through domino liver transplantation, using a donor liver graft from a patient with familial amyloid neuropathy (FAP), was first described in 2005.1 This complication had not been expected, given that the onset of neurologic symptoms in cases of inherited amyloidosis typically occurs in the age range of 25 to 40 years. Accelerated deposition of amyloid in these types of liver recipients may be facilitated by the presence of amyloid fibrils in the donor liver, which act as a template for amyloidogenesis. We present a case with an unusually short latency of symptomatic onset in which therapy with diflunisal may have helped stabilize neuropathy and nephropathy while the patient waited for another liver transplant.

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Figure.
Sural Nerve Sections With Transthyretin-Amyloid Deposits in the Endoneurium of a Nerve Fascicle

Scale bars = 50 μm, and the arrowheads indicate the transthyretin-amyloid deposits that were revealed after staining.

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