• Hae Il Cheong, Sang Kyung Jo, Sung Soo Yoon, Heeyeon Cho, Jin Seok Kim, Young Ok Kim, Ja Ryong Koo, Yong Park, Young Seo Park, Jae Il Shin, Kee Hwan Yoo, and Doyeun Oh.
• Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
• J. Korean Med. Sci. 2016 Oct 1; 31 (10): 1516-28.

AbstractAtypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by micro-angiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. The major pathogenesis of aHUS involves dysregulation of the complement system. Eculizumab, which blocks complement C5 activation, has recently been proven as an effective agent. Delayed diagnosis and treatment of aHUS can cause death or end-stage renal disease. Therefore, a diagnosis that differentiates aHUS from other forms of thrombotic microangiopathy is very important for appropriate management. These guidelines aim to offer recommendations for the diagnosis and treatment of patients with aHUS in Korea. The guidelines have largely been adopted from the current guidelines due to the lack of evidence concerning the Korean population.

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