Amyloid Polyneuropathy and Myocardial Amyloidosis 10 Years after Domino Liver Transplantation from a Patient with a <i>Transthyretin</i> Ser50Arg Mutation
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- Matsushima Masaaki
- Department of Neurology, Hokkaido University Graduate School of Medicine, Japan
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- Yabe Ichiro
- Department of Neurology, Hokkaido University Graduate School of Medicine, Japan
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- Tsuda Masaya
- Department of Cardiology, Hokkaido University Graduate School of Medicine, Japan
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- Sakakibara Mamoru
- Department of Cardiology, Hokkaido University Graduate School of Medicine, Japan
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- Shimamura Tsuyoshi
- Division of Organ Transplantation, Hokkaido University Hospital, Japan
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- Sasaki Hidenao
- Department of Neurology, Hokkaido University Graduate School of Medicine, Japan
Abstract
<p>A 54-year-old man with polycystic liver disease received a domino liver transplantation (DLT) from a patient of hereditary ATTR amyloidosis with the transthyretin Ser50Arg mutation. Ten years after transplantation, he felt a slight numbness in his toes, and cardiac amyloidosis was simultaneously suspected upon a heart function evaluation. Biopsy specimens from the myocardium revealed transthyretin amyloidosis with the Ser50Arg mutation. Oral tafamidis therapy has inhibited the progression of neurological and cardiovascular symptoms this far. We herein report this first case of amyloid polyneuropathy and myocardial amyloidosis after DLT from hereditary ATTR amyloidosis with a transthyretin Ser50Arg mutation and discuss similar cases of other mutations. </p>
Journal
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- Internal Medicine
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Internal Medicine 56 (23), 3231-3235, 2017
The Japanese Society of Internal Medicine