A case of erythropoietic protoporphyria with severe liver damage

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  • Shimazaki Hiroaki
    Department of Liver, Biliary Tract and Pancreas Diseases, Fujita Health University
  • Arima Yuko
    Department of Liver, Biliary Tract and Pancreas Diseases, Fujita Health University
  • Nakano Takuji
    Department of Liver, Biliary Tract and Pancreas Diseases, Fujita Health University
  • Murao Michihito
    Department of Liver, Biliary Tract and Pancreas Diseases, Fujita Health University
  • Nitta Yoshifumi
    Department of Liver, Biliary Tract and Pancreas Diseases, Fujita Health University
  • Harata Masao
    Department of Liver, Biliary Tract and Pancreas Diseases, Fujita Health University
  • Kawabe Naoto
    Department of Liver, Biliary Tract and Pancreas Diseases, Fujita Health University
  • Hashimoto Senju
    Department of Liver, Biliary Tract and Pancreas Diseases, Fujita Health University
  • Nagano Kenichi
    Department of Gastroenterology, Meijo Hospital
  • Ishikawa Tetsuya
    Department of Gastroenterology, Nagoya Kyoritsu Hospital
  • Okumura Akihiko
    Department of Gastroenterology, Kainan Hospital
  • Hayashi Kazuhiko
    Department of Gastroenterology, Nagoya University
  • Katano Yoshiaki
    Department of Gastroenterology, Nagoya University
  • Kuroda Makoto
    Department of Diagnostic Pathology, Fujita Health University
  • Yoshioka Kentaro
    Department of Liver, Biliary Tract and Pancreas Diseases, Fujita Health University

Bibliographic Information

Other Title
  • 著明な肝障害を伴ったプロトポルフィリン症の１例
  • 症例報告 著明な肝障害を伴ったプロトポルフィリン症の1例
  • ショウレイ ホウコク チョメイ ナ カン ショウガイ オ トモナッタ プロトポルフィリンショウ ノ 1レイ

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Abstract

Case is a 29-yr-old man, whose complaint was abdominal pain. He had photosensitivity since childhood. The paternal grandfather and the younger brother have photosensitivity. In November 2001, he admitted in the other hospital with liver dysfunction and was diagnosed as erythropoietic protoporphyria (EPP). In August 2005, he admitted with abdominal pain and jaundice and recovered with antibiotics. In January 2006, he was referred to our hospital with mild elevation of ALT. In November 2006 and in May 2007, he admitted in our hospital with abdominal pain and jaundice, and recovered with antibiotics. In August 2008, he admitted with the same symptoms. Erythrocyte protoporphyrin and total bilirubin continually elevated. He died from liver failure in November 2008. A small portion of EPP patients suffer from liver failure. The only effective treatment for liver failure of EPP is liver transplantation, which should be considered at appropriate timing of the disease course.<br>

Journal

  • Kanzo

    Kanzo 51 (4), 175-182, 2010

    The Japan Society of Hepatology

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