妊娠、肾移植或高血压急症相关的非典型溶血性尿毒症综合征的最新资料

Khalil El Karoui
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摘要

血栓性微血管病变(TMA)是一组异质性疾病,其特征不同程度上以机械性溶血性贫血、血小板减少症和组织学微血管闭塞为特征。TMA可并发多器官疾病,主要是神经和肾病(定义溶血性尿毒症综合征,HUS)。基于疾病病理生理学的分类强调了非典型(非感染相关)溶血性尿毒综合征中替代补体途径失调的重要性。然而,在特定情况下(如妊娠、肾移植或高血压急诊),许多形式的aHUS的病理生理学和治疗仍然缺乏特征。在本文中,我们回顾了这三种形式的aHUS在特定情况下的最新诊断和治疗进展。我们展示了这些形式的特殊性,并描述了其管理的当前和未来的挑战。
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Recent data on atypical hemolytic uremic syndrome associated with pregnancy, kidney transplantation or hypertensive emergency

Thrombotic microangiopathies (TMA) are a heterogeneous group of disorders characterized to varying degrees by mechanical hemolytic anemia, thrombocytopenia and histological microvascular occlusion. TMA can be complicated by multiple organ disorders, mainly neurological and nephrological (defining the hemolytic uremic syndrome, HUS). Classification based on disease pathophysiology has highlighted the importance of dysregulation of the alternative complement pathway in atypical (non-infection-related) HUS. However, the pathophysiology and treatment of many forms of aHUS in specific contexts (such as pregnancy, renal transplantation or hypertensive emergencies) remain poorly characterized. In this article, we review recent diagnostic and therapeutic advances in these three forms of aHUS in specific contexts. We show the specificities of these forms and describe the current and future challenges of their management.

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