Servicio de Nefrología, Hospital Vall d´Hebrón, Barcelona, Spain.
Unidad de Investigacio´n, Hospital La Paz, Madrid, Spain.
Clin Kidney J. 2012 Feb;5(1):31-3. doi: 10.1093/ndtplus/sfr157. Epub 2012 Jan 31.
Atypical haemolytic uraemic syndrome (aHUS) is a rare and life-threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy. We report the case of an adult patient with plasmaphaeresis-resistant aHUS and hypertension treated with the complement inhibitor eculizumab. Eculizumab was shown to completely inhibit haemolysis, normalize thrombocyte levels and increase diuresis. Full recovery of renal function was not possible due to irreversible renal damage prior to eculizumab initiation. These findings highlight the importance of early treatment with eculizumab in patients with poor response to standard therapy, in order to avoid irreversible renal damage.
非典型溶血尿毒症综合征（aHUS）是一种罕见且危及生命的疾病，由补体系统失调引起，导致不受控制的补体激活和血栓性微血管病。我们报告了一例对血浆置换有抗性的 aHUS 合并高血压的成年患者，该患者接受了补体抑制剂依库珠单抗治疗。依库珠单抗可完全抑制溶血、使血小板计数正常化并增加利尿。由于在开始依库珠单抗治疗之前已发生不可逆转的肾损伤，肾功能无法完全恢复。这些发现强调了在对标准治疗反应不佳的患者中早期使用依库珠单抗治疗的重要性，以避免不可逆转的肾损伤。
