Clinical guides for atypical hemolytic uremic syndrome in Japan
Clinical guides for atypical hemolytic uremic syndrome in Japan
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DOI：
10.1007/s10157-016-1276-6
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发表时间：
2016-08-01
影响因子：
2.3
通讯作者：
Kagami, Shoji
中科院分区：
文献类型：
--
作者：
Kato, Hideki;Nangaku, Masaomi;Kagami, Shoji
非典型溶血性尿毒症综合征(AHUS)是一种罕见的疾病，以微血管病理性溶血性贫血、血小板减少和急性肾损伤三者为特征。2013年，我们制定了诊断标准，以实现对aHUS的早期诊断和及时启动适当的治疗。最近的临床和分子发现导致了血栓性微血管病和aHUS的几种拟议的分类和定义。基于这一领域的最新进展和国际上正在形成的将继发性TMA排除在aHUS定义之外的共识，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。
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.