Clinical guides for atypical hemolytic uremic syndrome in Japan

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• 作者：Hideki Kato ; Masaomi Nangaku ; Hiroshi Hataya ; Toshihiro Sawai ; Akira Ashida ; Rika Fujimaru ; Yoshihiko Hidaka ; Shinya Kaname ; Shoichi Maruyama ; Takashi Yasuda ; Yoko Yoshida ; Shuichi Ito ; Motoshi Hattori ; Yoshitaka Miyakawa ; Yoshihiro Fujimura ; Hirokazu Okada ; Shoji Kagami and The Joint Committee for the Revision of Clinical Guides of Atypical Hemolytic Uremic Syndrome in Japan
• 刊名：Pediatrics International
• 出版年：2016
• 出版时间：July 2016
• 年：2016
• 卷：58
• 期：7
• 页码：549-555
• 全文大小：120K
• ISSN：1442-200X

文摘

Atypical hemolytic uremic syndrome (aHUS) 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 aHUS. Recent clinical and molecular findings have resulted in several proposed classifications and definitions of thrombotic microangiopathy and aHUS. Based on recent advances in this field and the emerging international consensus to exclude secondary TMAs from the definition of aHUS, we have redefined aHUS and proposed diagnostic algorithms, differential diagnosis, and therapeutic strategies for aHUS.