A case of renal transplantation with Fabry disease after introduction of enzyme replacement therapy

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  • Ishikawa Yasunobu
    Department of Internal Medicine, Graduate School of Medicine, Hokkaido University
  • Shibazaki Sekiya
    Department of Internal Medicine, Graduate School of Medicine, Hokkaido University
  • Ito Masanori
    Department of Internal Medicine, Graduate School of Medicine, Hokkaido University
  • Nishimura Sayoko
    Department of Internal Medicine, Graduate School of Medicine, Hokkaido University
  • Maoka Tomochika
    Department of Internal Medicine, Graduate School of Medicine, Hokkaido University
  • Ebata Shinichi
    Department of Internal Medicine, Graduate School of Medicine, Hokkaido University
  • Shimizu Jun
    Department of Internal Medicine, Graduate School of Medicine, Hokkaido University
  • Hashimoto Seiji
    Department of Internal Medicine, Graduate School of Medicine, Hokkaido University
  • Morita Ken
    Department of Renal and Genitourinary Surgery, Graduate School of Medicine, Hokkaido University
  • Watarai Yoshihiko
    Department of Renal and Genitourinary Surgery, Graduate School of Medicine, Hokkaido University
  • Mochizuki Toshio
    Department of Internal Medicine, Graduate School of Medicine, Hokkaido University
  • Nonomura Katsuya
    Department of Renal and Genitourinary Surgery, Graduate School of Medicine, Hokkaido University
  • Koike Takao
    Department of Internal Medicine, Graduate School of Medicine, Hokkaido University

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  • α‐ｇａｌａｃｔｏｓｉｄａｓｅ　Ａの酵素補充療法を開始後に腎移植を施行したＦａｂｒｙ病の１例
  • ショウレイ ホウコク アルファ galactosidase A ノ コウソ ホジュウ リョウホウ オ カイシ ゴ ニ ジンイショク オ シコウ シタ Fabryビョウ ノ 1レイ

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Abstract

This is the first case of renal transplantation with Fabry disease after introduction of enzyme replacement therapy (ERT). A 32-year-old man, who was diagnosed Fabry disease at the age of 13, was referred to our hospital because of chronic renal failure. To prevent the further progression of renal dysfunction, ERT of α-galactosidase A was introduced. Although plasma and urinary concentration of globotriaosylceramide (GL-3) were reduced significantly, renal function deteriorated gradually and reached the level of end stage renal disease. Two weeks after initiating hemodialysis, he underwent living renal transplantation with his father as a donor. Graft kidney functioned well, and ERT was continued. ERT of α-galactosidase A after renal transplantation is considerable for patients with Fabry disease to prevent not only accumulation of GL-3 in graft kidney but also cardiovascular disease as a fatal complication of Fabry disease. In renal transplant patients, the indicator for ERT should not be urinary GL-3 but plasma GL-3.

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