Clinical guides for atypical hemolytic uremic syndrome in Japan

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  • Hideki Kato
    Division of Nephrology and Endocrinology The University of Tokyo Graduate School of Medicine Tokyo Japan
  • Masaomi Nangaku
    Division of Nephrology and Endocrinology The University of Tokyo Graduate School of Medicine Tokyo Japan
  • Hiroshi Hataya
    Department of Nephrology Tokyo Metropolitan Children's Medical Center Fuchu Tokyo Japan
  • Toshihiro Sawai
    Department of Pediatrics Shiga University of Medical Science Otsu Shiga Japan
  • Akira Ashida
    Department of Pediatrics Osaka Medical College Takatsuki Osaka Japan
  • Rika Fujimaru
    Department of Pediatrics Osaka City General Hospital Miyakojima Osaka Japan
  • Yoshihiko Hidaka
    Department of Pediatrics Shinshu University School of Medicine Matsumoto Nagano Japan
  • Shinya Kaname
    First Department of Internal Medicine Kyorin University School of Medicine Mitaka Tokyo Japan
  • Shoichi Maruyama
    Department of Nephrology Nagoya University Graduate School of Medicine Nagoya Aichi Japan
  • Takashi Yasuda
    Kichijoji Asahi Hospital Musashino Tokyo Japan
  • Yoko Yoshida
    Division of Nephrology and Endocrinology The University of Tokyo Graduate School of Medicine Tokyo Japan
  • Shuichi Ito
    Department of Pediatrics Graduate School of Medicine Yokohama City University Kanazawa Yokohama Japan
  • Motoshi Hattori
    Department of Pediatric Nephrology Tokyo Women's Medical University Shinjuku Tokyo Japan
  • Yoshitaka Miyakawa
    Department of General Internal Medicine Faculty of Medicine Saitama Medical University Iruma Saitama Japan
  • Yoshihiro Fujimura
    Department of Blood Transfusion Medicine Nara Medical University Kashihara Nara Japan
  • Hirokazu Okada
    Department of Nephrology Faculty of Medicine Saitama Medical University Iruma Saitama Japan
  • Shoji Kagami
    Department of Pediatrics Graduate School of Medical Sciences Tokushima University Tokushima Japan

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<jats:title>Abstract</jats:title><jats:p>Atypical hemolytic uremic syndrome (<jats:styled-content style="fixed-case">aHUS</jats:styled-content>) is a rare disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. In 2013, we developed diagnostic criteria to enable early diagnosis and timely initiation of appropriate treatment for <jats:styled-content style="fixed-case">aHUS</jats:styled-content>. Recent clinical and molecular findings have resulted in several proposed classifications and definitions of thrombotic microangiopathy and <jats:styled-content style="fixed-case">aHUS</jats:styled-content>. Based on recent advances in this field and the emerging international consensus to exclude secondary <jats:styled-content style="fixed-case">TMA</jats:styled-content>s from the definition of <jats:styled-content style="fixed-case">aHUS</jats:styled-content>, we have redefined <jats:styled-content style="fixed-case">aHUS</jats:styled-content> and proposed diagnostic algorithms, differential diagnosis, and therapeutic strategies for <jats:styled-content style="fixed-case">aHUS</jats:styled-content>.</jats:p>

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