| Affiliation: | 1.Division of Nephrology and Endocrinology,The University of Tokyo Graduate School of Medicine,Bunkyo,Japan;2.Department of Nephrology,Tokyo Metropolitan Children’s Medical Center,Fuchu,Japan;3.Department of Pediatrics,Shiga University of Medical Science,Otsu,Japan;4.Department of Pediatrics,Osaka Medical College,Takatsuki,Japan;5.Department of Pediatrics,Osaka City General Hospital,Miyakojima,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,Nagoya University Graduate School of Medicine,Nagoya,Japan;9.Kichijoji Asahi Hospital,Musashino,Japan;10.Department of Pediatrics, Graduate School of Medicine,Yokohama City University,Kanazawa,Japan;11.Department of Pediatric Nephrology,Tokyo Women’s Medical University,Shinjuku,Japan;12.Department of General Internal Medicine, Faculty of Medicine,Saitama Medical University,Iruma,Japan;13.Department of Blood Transfusion Medicine,Nara Medical University,Kashihara,Japan;14.Department of Nephrology, Faculty of Medicine,Saitama Medical University,Iruma,Japan;15.Department of Pediatrics, Graduate School of Medical Sciences,Tokushima University,Tokushima,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. |
