Two recovery cases of Wilson's disease initiated with severe hemolysis and acute hepatic failure

  • TAKAHASHI Atsushi
    Department of Internal Medicine II, Fukushima Medical University School of Medicine
  • SUZUKI Tomohiro
    Department of Internal Medicine II, Fukushima Medical University School of Medicine
  • SHISHIDO Shoichiro
    Department of Internal Medicine II, Fukushima Medical University School of Medicine
  • TOJO Jun
    Department of Internal Medicine II, Fukushima Medical University School of Medicine
  • ITO Osamu
    Department of Internal Medicine II, Fukushima Medical University School of Medicine
  • KAZUTA Yoshihiro
    Department of Internal Medicine II, Fukushima Medical University School of Medicine
  • WATANABE Hiroshi
    Department of Internal Medicine II, Fukushima Medical University School of Medicine
  • ISHIKAWA Hidemasa
    Department of Internal Medicine II, Fukushima Medical University School of Medicine
  • OHIRA Hiromasa
    Department of Internal Medicine II, Fukushima Medical University School of Medicine
  • OBARA Katsutoshi
    Department of Internal Medicine II, Fukushima Medical University School of Medicine
  • KASUKAWA Reiji
    Department of Internal Medicine II, Fukushima Medical University School of Medicine

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  • 溶血発作で発症し肝不全症状を呈したが救命し得たWilson病の2例

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症例1は13歳の男性.肝機能障害と溶血性貧血で発症.血清セルロプラスミン値の低下,尿中銅排泄量の増加,Kayser-Fleischer輪からWilson病と診断されD-ぺニシラミンを投与された.しかしビリルビンの上昇と意識障害が出現し,血漿交換療法を施行され改善した.症例2は15歳の女性.黄疸と溶血性貧血で発症.血清セルロプラスミン値の低下,尿中銅排泄量の増加,Kayser-Fleischer輪からWilson病と診断され,D-ぺニシラミンの投与と血漿交換療法を施行され一時改善.しかしD-ぺニシラミンから塩酸トリエンチンへの変更にともない再び溶血性貧血が出現し,再度血漿交換療法を施行され塩酸トリエンチンの増量にて改善が得られた,溶血発作・肝不全症状で発症し内科的治療が奏功したWilson病の2例を報告した.

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