妊娠相关性非典型溶血性尿毒症综合征伴肾脏恢复，尽管延迟启动补体阻断
Pregnancy-Associated Atypical Hemolytic Uremic Syndrome With Kidney Recovery Despite Delayed Initiation of Complement Blockade.
摘要(中文译文)
非典型溶血性尿毒症综合征（aHUS）是一种补体介导的血栓性微血管病（TMA），以微血管病性溶血性贫血、血小板减少和急性肾损伤为特征，妊娠是公认的触发因素，尤其是在妊娠晚期和产后期。我们报告一例29岁初产妇，妊娠33周，表现为严重急性肾损伤（AKI）、血小板减少、贫血、乳酸脱氢酶升高及高胆红素血症，而肝酶正常。ADAMTS13活性正常，自身免疫血清学阴性。她需要紧急血液透析，并因母体指征行急诊剖宫产术；然而产后肾功能衰竭持续存在，依赖透析。肾活检显示血栓性微血管病伴小动脉纤维蛋白血栓。基因检测发现补体因子H存在杂合致病性剪接位点变异，支持补体介导性aHUS的诊断。在就诊约五周后开始使用依库珠单抗进行补体抑制治疗，后转换为雷夫利珠单抗。尽管治疗延迟且初始依赖透析，她在开始补体阻断治疗五周后实现透析独立，肾功能部分恢复，血小板计数恢复正常。本病例强调，对于严重肾功能障碍与肝酶异常不成比例的妊娠患者，需考虑补体介导性TMA，并表明即使需要透析且未立即开始补体抑制治疗，仍有意义的肾功能恢复仍有可能。
Abstract
Atypical hemolytic uremic syndrome (aHUS) is a complement-mediated thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, and pregnancy is a recognized trigger, particularly in the third trimester and postpartum period. We report a 29-year-old primigravida at 33 weeks' gestation who presented with severe acute kidney injury (AKI), thrombocytopenia, anemia, elevated lactate dehydrogenase, and hyperbilirubinemia with otherwise normal liver enzymes. The ADAMTS13 activity was normal, and autoimmune serologies were negative. She required urgent hemodialysis and underwent emergency cesarean section for maternal indications; however, renal failure persisted postpartum with dialysis dependence. Kidney biopsy demonstrated thrombotic microangiopathy with arteriolar fibrin thrombi. Genetic testing identified a heterozygous pathogenic splice-site variant in complement factor H, supporting the diagnosis of complement-mediated aHUS. Complement inhibition with eculizumab was initiated approximately five weeks after presentation and later transitioned to ravulizumab. Despite delayed therapy and initial dialysis dependence, she achieved dialysis independence five weeks after initiation of complement blockade, with partial renal recovery and normalization of platelet count. This case emphasizes the need to consider complement-mediated TMA in pregnant patients with severe renal dysfunction disproportionate to liver enzyme abnormalities and demonstrates that meaningful renal recovery remains possible even when dialysis is required, and complement inhibition is not initiated immediately.
如何引用
AMA
Abdurrahman Hamadah, Tarek Eleraky, Dileep Kumar, Dhanya Mohan, Aisha Bibi, Herlitz Leal, et al. Pregnancy-Associated Atypical Hemolytic Uremic Syndrome With Kidney Recovery Despite Delayed Initiation of Complement Blockade.. Cureus. 2026; doi:10.7759/cureus.108064.
APA
Abdurrahman Hamadah, Tarek Eleraky, Dileep Kumar, Dhanya Mohan, Aisha Bibi, Herlitz Leal, et al (2026). Pregnancy-Associated Atypical Hemolytic Uremic Syndrome With Kidney Recovery Despite Delayed Initiation of Complement Blockade.. Cureus. https://doi.org/10.7759/cureus.108064
GB/T 7714
Abdurrahman Hamadah, Tarek Eleraky, Dileep Kumar, Dhanya Mohan, Aisha Bibi, Herlitz Leal, et al. Pregnancy-Associated Atypical Hemolytic Uremic Syndrome With Kidney Recovery Despite Delayed Initiation of Complement Blockade.[J]. Cureus, 2026 doi:10.7759/cureus.108064.
在 PubMed增强版 阅读全文
翻译全文 · AI 文献问答 · PDF 原文下载 · 引用导出