Department of Nephrology, Children's Hospital of Pittsburgh of UPMC, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Pediatr Nephrol. 2019 Aug;34(8):1337-1348. doi: 10.1007/s00467-018-4039-7. Epub 2018 Aug 14.
Atypical hemolytic uremic syndrome (aHUS) is a rare and complex disease resulting from abnormal alternative complement activation with a wide range of clinical presentations. Extra-renal manifestations of aHUS can involve many organ systems, including the peripheral and central nervous, gastrointestinal, cardiovascular, integumentary, pulmonary, as well as the eye. While some of these extra-renal manifestations occur in the acute phase of aHUS, some can also occur as long-term sequelae of unopposed complement activation. Extra-renal symptoms are observed in approximately 20% of patients with aHUS, with the incidence of specific organ system complications ranging from a few case reports to 50% of described patients. Careful monitoring for extra-renal involvement is critical in patients with aHUS, as prompt evaluation and management may decrease the risk of high morbidity and mortality associated with aHUS.
非典型溶血尿毒综合征（aHUS）是一种罕见且复杂的疾病，由异常替代补体激活引起，临床表现多样。aHUS 的肾外表现可累及多个器官系统，包括外周和中枢神经系统、胃肠道、心血管系统、皮肤、肺以及眼睛。虽然这些肾外表现中的一些发生在 aHUS 的急性期，但也有一些可能是由于补体激活未被抑制而作为长期后遗症出现。大约 20%的 aHUS 患者存在肾外症状，特定器官系统并发症的发生率从少数病例报告到描述的患者的 50%不等。仔细监测 aHUS 患者的肾外表现至关重要，因为及时评估和管理可能会降低与 aHUS 相关的高发病率和死亡率的风险。
