Clinical guides for atypical hemolytic uremic syndrome in Japan

Hideki Kato¹, Masaomi Nangaku¹, Hiroshi Hataya², Toshihiro Sawai³, Akira Ashida⁴, Rika Fujimaru⁵, Yoshihiko Hidaka⁶, Shinya Kaname⁷, Shoichi Maruyama⁸, Takashi Yasuda⁹, Yoko Yoshida¹, Shuichi Ito¹⁰, Motoshi Hattori¹¹, Yoshitaka Miyakawa¹², Yoshihiro Fujimura¹³, Hirokazu Okada¹⁴, Shoji Kagami¹⁵; Joint Committee for the Revision of Clinical Guides of Atypical Hemolytic Uremic Syndrome in Japan

Affiliations

¹ Division of Nephrology and Endocrinology, The University of Tokyo Graduate School of Medicine, Bunkyo, Tokyo, Japan.
² Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Fuchu, Tokyo, Japan.
³ Department of Pediatrics, Shiga University of Medical Science, Otsu, Shiga, Japan.
⁴ Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan.
⁵ Department of Pediatrics, Osaka City General Hospital, Miyakojima, Osaka, Japan.
⁶ Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
⁷ First Department of Internal Medicine, Kyorin University School of Medicine, Mitaka, Tokyo, Japan.
⁸ Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.
⁹ Kichijoji Asahi Hospital, Musashino, Tokyo, Japan.
¹⁰ Department of Pediatrics, Graduate School of Medicine, Yokohama City University, Kanazawa, Yokohama, Japan.
¹¹ Department of Pediatric Nephrology, Tokyo Women's Medical University, Shinjuku, Tokyo, Japan.
¹² Department of General Internal Medicine, Faculty of Medicine, Saitama Medical University, Iruma, Saitama, Japan.
¹³ Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Nara, Japan.
¹⁴ Department of Nephrology, Faculty of Medicine, Saitama Medical University, Iruma, Saitama, Japan.
¹⁵ 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.

PMID: 27422619
DOI: 10.1007/s10157-016-1276-6

Practice Guideline

Clinical guides for atypical hemolytic uremic syndrome in Japan

Hideki Kato et al. Clin Exp Nephrol. 2016 Aug.

. 2016 Aug;20(4):536-543.

doi: 10.1007/s10157-016-1276-6.

Authors

Affiliations

¹ Division of Nephrology and Endocrinology, The University of Tokyo Graduate School of Medicine, Bunkyo, Tokyo, Japan.
² Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Fuchu, Tokyo, Japan.
³ Department of Pediatrics, Shiga University of Medical Science, Otsu, Shiga, Japan.
⁴ Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan.
⁵ Department of Pediatrics, Osaka City General Hospital, Miyakojima, Osaka, Japan.
⁶ Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
⁷ First Department of Internal Medicine, Kyorin University School of Medicine, Mitaka, Tokyo, Japan.
⁸ Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.
⁹ Kichijoji Asahi Hospital, Musashino, Tokyo, Japan.
¹⁰ Department of Pediatrics, Graduate School of Medicine, Yokohama City University, Kanazawa, Yokohama, Japan.
¹¹ Department of Pediatric Nephrology, Tokyo Women's Medical University, Shinjuku, Tokyo, Japan.
¹² Department of General Internal Medicine, Faculty of Medicine, Saitama Medical University, Iruma, Saitama, Japan.
¹³ Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Nara, Japan.
¹⁴ Department of Nephrology, Faculty of Medicine, Saitama Medical University, Iruma, Saitama, Japan.
¹⁵ 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.

PMID: 27422619
DOI: 10.1007/s10157-016-1276-6

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.

Keywords: Alternative complement pathway; Atypical hemolytic uremic syndrome; Eculizumab; Thrombotic microangiopathy.

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References

1. Nefrologia. 2013 Jan 18;33(1):27-45 - PubMed
1. Br J Haematol. 2014 Mar;164(6):759-66 - PubMed
1. J Am Soc Nephrol. 2010 May;21(5):859-67 - PubMed
1. Blood. 2013 Sep 19;122(12):2003-7 - PubMed
1. Nihon Jinzo Gakkai Shi. 2014;56(7):1043-51 - PubMed

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Clinical guides for atypical hemolytic uremic syndrome in Japan

Affiliations

Clinical guides for atypical hemolytic uremic syndrome in Japan

Authors

Affiliations

Abstract

References

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MeSH terms

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