Umscheid Jacob H, Nevil Collin, Vasudeva Rhythm, Ali Mohammed Farhan, Agasthya Nisha
University of Kansas, School of Medicine, Wichita, USA.
Children's Mercy Hospital, Department of Nephrology, Kansas City, USA.
Case Rep Pediatr. 2021 Nov 13;2021:8053246. doi: 10.1155/2021/8053246. eCollection 2021.
Hemolytic Uremic Syndrome (HUS) is a constellation of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Shiga toxin-producing - (STEC-) mediated HUS is a common cause of acute renal failure in children and can rarely result in severe neurological complications such as encephalopathy, seizures, cerebrovascular accidents, and coma. Current literature supports use of eculizumab, a monoclonal antibody that blocks complement activation, in atypical HUS (aHUS). However, those with neurologic complications from STEC-HUS have complement activation and deposition of aggregates in microvasculature and may be treated with eculizumab. In this case report, we describe a 3-year-old boy with diarrhea-positive STEC-HUS who developed severe neurologic involvement in addition to acute renal failure requiring renal replacement therapy. He was initiated on eculizumab therapy, with clinical improvement and organ recovery. This case highlights systemic complications of STEC-HUS in a pediatric patient. The current literature is limited but has suggested a role for complement mediation in cases with severe complications. We review the importance of early recognition of complications, use of eculizumab, and current data available.
溶血尿毒综合征（HUS）是一种微血管病性溶血性贫血、血小板减少症和急性肾衰竭的综合征。产志贺毒素大肠杆菌（STEC）介导的HUS是儿童急性肾衰竭的常见原因，很少会导致严重的神经系统并发症，如脑病、癫痫发作、脑血管意外和昏迷。目前的文献支持在非典型HUS（aHUS）中使用依库珠单抗，这是一种阻断补体激活的单克隆抗体。然而，那些患有STEC-HUS神经系统并发症的患者存在补体激活和微血管中聚集体沉积的情况，可能可用依库珠单抗治疗。在本病例报告中，我们描述了一名3岁腹泻阳性STEC-HUS男孩，除了需要肾脏替代治疗的急性肾衰竭外，还出现了严重的神经系统受累。他开始接受依库珠单抗治疗，临床症状改善，器官功能恢复。本病例突出了儿科患者中STEC-HUS的全身并发症。目前的文献有限，但已表明补体介导在严重并发症病例中的作用。我们回顾了早期识别并发症、使用依库珠单抗的重要性以及现有数据。
