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首页> 外文期刊>Clinical kidney journal. >Eculizumab in atypical haemolytic–uraemic syndrome allows cessation of plasma exchange and dialysis
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Eculizumab in atypical haemolytic–uraemic syndrome allows cessation of plasma exchange and dialysis

机译：非典型溶血尿毒综合症中的依库丽单抗可停止血浆交换和透析

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Disorders in complement regulation are a major cause of atypical haemolytic–uraemic syndrome (aHUS). Eculizumab, a monoclonal antibody targeting complement C5 and blocking the terminal complement cascade, should theoretically be useful in this disease, particularly when associated with specific complement pathway anomalies such as Factor H deficiency. Eculizumab is emerging as an effective treatment for post-transplant aHUS recurrence and may have a role in treating de novo aHUS, halting the haemolytic process. In this case report, we describe the fourth case of aHUS treated with eculizumab. In our patient, with a known complement Factor H mutation, not only has the disease process become quiescent but also this therapy has led to significantly improved renal function so that dialysis is no longer necessary.
机译：补体调节紊乱是非典型溶血尿毒综合症（aHUS）的主要原因。 Eculizumab是靶向补体C5并阻断末端补体级联反应的单克隆抗体，在理论上应在该疾病中有用，特别是与特定的补体途径异常（例如H因子缺乏症）相关时。依库丽单抗正在成为一种有效的移植后aHUS复发治疗方法，并且可能在治疗aHUS从头开始，中止溶血过程中发挥作用。在此病例报告中，我们描述了使用依库丽单抗治疗的aHUS的第四例。在我们的患者中，具有已知的补体因子H突变，不仅使疾病过程变得静止，而且这种疗法已导致肾功能显着改善，因此不再需要透析。

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