Kato Hideki, Nangaku Masaomi, Hataya Hiroshi, Sawai Toshihiro, Ashida Akira, Fujimaru Rika, Hidaka Yoshihiko, Kaname Shinya, Maruyama Shoichi, Yasuda Takashi, Yoshida Yoko, Ito Shuichi, Hattori Motoshi, Miyakawa Yoshitaka, Fujimura Yoshihiro, Okada Hirokazu, Kagami Shoji
Division of Nephrology and Endocrinology, The University of Tokyo Graduate School of Medicine, Tokyo, Japan.
Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Fuchu, Tokyo, Japan.
Pediatr Int. 2016 Jul;58(7):549-55. doi: 10.1111/ped.13044.
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.
非典型溶血尿毒综合征（aHUS）是一种罕见疾病，其特征为微血管病性溶血性贫血、血小板减少和急性肾损伤三联征。2013年，我们制定了诊断标准，以便对aHUS进行早期诊断并及时开始适当治疗。最近的临床和分子研究结果促成了血栓性微血管病和aHUS的几种分类及定义。基于该领域的最新进展以及将继发性血栓性微血管病排除在aHUS定义之外的新出现的国际共识，我们重新定义了aHUS，并提出了aHUS的诊断算法、鉴别诊断和治疗策略。
