• Internal medicine · Aug 2022

    Case Reports Observational Study

    Deterioration after Liver Transplantation and Transthyretin Stabilizer Administration in a Patient with ATTRv Amyloidosis with a Leu58Arg (p.Leu78Arg) TTR Variant.

    • Sadao Hikishima, Kenji Sakai, Akio Akagi, Hiroki Yamaguchi, Shutaro Shibata, Koji Hayashi, Hiroto Nakano, Mizuki Kanemoto, Yuta Usui, Yu Taniguchi, Junji Komatsu, Keiko Nakamura-Shindo, Ichiro Nozaki, Tsuyoshi Hamaguchi, Kenjiro Ono, Kazuo Iwasa, and Masahito Yamada.
    • Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences, Japan.
    • Intern. Med. 2022 Aug 1; 61 (15): 2347-2351.

    AbstractWe herein report a 44-year-old Japanese man with hereditary transthyretin amyloidosis (ATTRv amyloidosis) harboring the variant Leu58Arg (p.Leu78Arg) in TTR in whom we conducted an observational study with liver transplantation (LT) and transthyretin (TTR) stabilizers (tafamidis and diflunisal) for 9 years. This patient showed gradual deterioration of sensory, motor, and autonomic neuropathy symptoms after LT. Furthermore, cardiac amyloidosis gradually developed. Although the present case showed deterioration of the symptoms after disease-modifying treatments, LT might be suitable in patients with the same variant if they are young and in good condition due to a long survival after LT.

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