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Transthyretin-type cerebral amyloid angiopathy: a serious complication in post-transplant patients with familial amyloid polyneuropathy
  1. Yoshiki Sekijima1,2
  1. 1Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan
  2. 2Institute for Biomedical Sciences, Shinshu University, Matsumoto, Japan
  1. Correspondence to Dr Yoshiki Sekijima, Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan; sekijima{at}shinshu-u.ac.jp

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Liver transplantation is a well-established treatment for transthyretin (TTR)-type familial amyloid polyneuropathy (TTR-FAP).1 According to data in the Familial Amyloidotic Polyneuropathy World Transplant Registry (http://www.fapwtr.org/ram_fap.htm), more than 2000 liver transplantations have been performed to date in 19 countries. Transplantation replaces the variant TTR gene with the wild-type gene in the liver, the main source of serum circulating TTR. The serum concentration of variant TTR decreases rapidly, reaching almost zero after the operation. The effects of liver transplantation on neuropathy are evident as its progression is …

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