title: Clinical guides for atypical hemolytic uremic syndrome in Japan.
abstract: 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.
authors.affiliation: Division of Nephrology and Endocrinology, The University of Tokyo Graduate School of Medicine, Bunkyo, Tokyo, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Kato
authors.affiliation: Division of Nephrology and Endocrinology, The University of Tokyo Graduate School of Medicine, Bunkyo, Tokyo, Japan.
authors.forename: Masaomi
authors.initials: M
authors.lastname: Nangaku
authors.affiliation: Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Fuchu, Tokyo, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hataya
authors.affiliation: Department of Pediatrics, Shiga University of Medical Science, Otsu, Shiga, Japan.
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Sawai
authors.affiliation: Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Ashida
authors.affiliation: Department of Pediatrics, Osaka City General Hospital, Miyakojima, Osaka, Japan.
authors.forename: Rika
authors.initials: R
authors.lastname: Fujimaru
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Hidaka
authors.affiliation: First Department of Internal Medicine, Kyorin University School of Medicine, Mitaka, Tokyo, Japan.
authors.forename: Shinya
authors.initials: S
authors.lastname: Kaname
authors.affiliation: Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.
authors.forename: Shoichi
authors.initials: S
authors.lastname: Maruyama
authors.affiliation: Kichijoji Asahi Hospital, Musashino, Tokyo, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Yasuda
authors.affiliation: Division of Nephrology and Endocrinology, The University of Tokyo Graduate School of Medicine, Bunkyo, Tokyo, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Yoshida
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, Yokohama City University, Kanazawa, Yokohama, Japan.
authors.forename: Shuichi
authors.initials: S
authors.lastname: Ito
authors.affiliation: Department of Pediatric Nephrology, Tokyo Women's Medical University, Shinjuku, Tokyo, Japan.
authors.forename: Motoshi
authors.initials: M
authors.lastname: Hattori
authors.affiliation: Department of General Internal Medicine, Faculty of Medicine, Saitama Medical University, Iruma, Saitama, Japan.
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Miyakawa
authors.affiliation: Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Nara, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
authors.affiliation: Department of Nephrology, Faculty of Medicine, Saitama Medical University, Iruma, Saitama, Japan.
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Okada
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Tokushima University, 3-18-15 Kuramoto-cho, Tokushima, Tokushima, 770-8503, Japan. kagami@tokushima-u.ac.jp.
authors.forename: Shoji
authors.initials: S
authors.lastname: Kagami
mesh_terms: D065766:Atypical Hemolytic Uremic Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D006801:Humans; D007564:Japan
keywords: Alternative complement pathway; Atypical hemolytic uremic syndrome; Eculizumab; Thrombotic microangiopathy