Department of Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden.
J Intern Med. 2017 Feb;281(2):123-148. doi: 10.1111/joim.12546. Epub 2016 Oct 10.
Haemolytic uraemic syndrome (HUS) is defined by the simultaneous occurrence of nonimmune haemolytic anaemia, thrombocytopenia and acute renal failure. This leads to the pathological lesion termed thrombotic microangiopathy, which mainly affects the kidney, as well as other organs. HUS is associated with endothelial cell injury and platelet activation, although the underlying cause may differ. Most cases of HUS are associated with gastrointestinal infection with Shiga toxin-producing enterohaemorrhagic Escherichia coli (EHEC) strains. Atypical HUS (aHUS) is associated with complement dysregulation due to mutations or autoantibodies. In this review, we will describe the causes of HUS. In addition, we will review the clinical, pathological, haematological and biochemical features, epidemiology and pathogenetic mechanisms as well as the biochemical, microbiological, immunological and genetic investigations leading to diagnosis. Understanding the underlying mechanisms of the different subtypes of HUS enables tailoring of appropriate treatment and management. To date, there is no specific treatment for EHEC-associated HUS but patients benefit from supportive care, whereas patients with aHUS are effectively treated with anti-C5 antibody to prevent recurrences, both before and after renal transplantation.
溶血尿毒综合征（HUS）的定义为非免疫性溶血性贫血、血小板减少症和急性肾衰竭同时发生。这导致了血栓性微血管病的病理损伤，主要影响肾脏以及其他器官。HUS 与内皮细胞损伤和血小板激活有关，尽管潜在的原因可能不同。大多数 HUS 病例与产志贺毒素的肠出血性大肠杆菌（EHEC）菌株引起的胃肠道感染有关。非典型 HUS（aHUS）与补体失调有关，原因是基因突变或自身抗体。在这篇综述中，我们将描述 HUS 的病因。此外，我们将回顾其临床、病理、血液学和生化特征、流行病学和发病机制以及导致诊断的生化、微生物学、免疫学和遗传学研究。了解不同类型 HUS 的潜在机制有助于制定适当的治疗和管理方案。迄今为止，针对 EHEC 相关 HUS 尚无特异性治疗方法，但患者受益于支持性治疗，而 aHUS 患者在肾移植前后用抗 C5 抗体有效治疗以预防复发。
