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Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the joint committee of the Japanese society of nephrology and the Japan pediatric society |
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| Authors: | Toshihiro Sawai Masaomi Nangaku Akira Ashida Rika Fujimaru Hiroshi Hataya Yoshihiko Hidaka Shinya Kaname Hirokazu Okada Waichi Sato Takashi Yasuda Yoko Yoshida Yoshihiro Fujimura Motoshi Hattori Shoji Kagami |
| Affiliation: | 1. Department of Pediatrics, Shiga University of Medical Science, Otsu, Japan 2. Division of Nephrology and Endocrinology, The University of Tokyo School of Medicine, Tokyo, Japan 3. Department of Pediatrics, Osaka Medical College, Takatsuki, Japan 4. Department of Pediatrics, Osaka City General Hospital, Osaka, Japan 5. Department of Nephrology, Tokyo Metropolitan Children’s Medical Center, Fuchu, Japan 6. Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan 7. First Department of Internal Medicine, Kyorin University School of Medicine, Mitaka, Japan 8. Department of Nephrology, Faculty of Medicine, Saitama Medical University, Saitama, Japan 9. Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan 10. Division of Nephrology and Hypertension, Department of Medicine, St. Marianna University School of Medicine, Kawasaki, Japan 11. Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan 12. Department of Pediatric Nephrology, Tokyo Women’s Medical University, Tokyo, Japan 13. Department of Pediatrics, Graduate School of Medical Sciences, Tokushima University, Tokushima, Japan |
| Abstract: | Atypical hemolytic uremic syndrome (aHUS) is rare and comprises the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Recently, abnormalities in the mechanisms underlying complement regulation have been focused upon as causes of aHUS. The prognosis for patients who present with aHUS is very poor, with the first aHUS attack being associated with a mortality rate of ~25 %, and with ~50 % of cases resulting in end-stage renal disease requiring dialysis. If treatment is delayed, there is a high risk of this syndrome progressing to renal failure. Therefore, we have developed diagnostic criteria for aHUS to enable its early diagnosis and to facilitate the timely initiation of appropriate treatment. We hope these diagnostic criteria will be disseminated to as many clinicians as possible and that they will be used widely. |
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