Greenwood Gregory T
Nephrology Associates, PLLC, Winston-Salem, NC, USA.
Int Med Case Rep J. 2015 Oct 13;8:235-9. doi: 10.2147/IMCRJ.S90640. eCollection 2015.
Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by chronic, uncontrolled activation of the alternative complement pathway, leading to thrombotic microangiopathy. Renal impairment and progression to end-stage renal disease are common in untreated patients with aHUS, and extrarenal manifestations are being increasingly characterized in the literature. Ocular involvement remains rare in aHUS. This report describes a patient with aHUS with bilateral central retinal artery and vein occlusion, vitreous hemorrhage, and blindness in addition to renal impairment. The patient's hematologic and renal parameters and ocular manifestation improved following initiation of eculizumab therapy.
非典型溶血性尿毒症综合征(aHUS)是一种由替代补体途径慢性、不受控制的激活引起的罕见疾病,可导致血栓性微血管病。在未经治疗的aHUS患者中,肾功能损害和进展至终末期肾病很常见,且文献中对肾外表现的描述越来越多。aHUS患者眼部受累仍然罕见。本报告描述了一名患有aHUS的患者,除肾功能损害外,还出现双侧视网膜中央动脉和静脉阻塞、玻璃体积血和失明。在开始使用依库珠单抗治疗后,患者的血液学和肾脏参数以及眼部表现均有所改善。