Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review

被引:2
|
作者
Cadet, Bair [1 ]
Meshoyrer, Daniel [2 ]
Kim, Zae [1 ]
机构
[1] Nassau Univ, Med Ctr, Dept Internal Med, Div Nephrol, 2201 Hempstead Turnpike, E Meadow, NY 11554 USA
[2] Nassau Univ, Med Ctr, Dept Family Med, E Meadow, NY 11554 USA
来源
关键词
atypical hemolytic uremic syndrome; pregnancy; end-stage renal disease; THROMBOTIC THROMBOCYTOPENIC PURPURA; DISSEMINATED INTRAVASCULAR COAGULATION; THERAPEUTIC APHERESIS; WRITING COMMITTEE; AMERICAN SOCIETY; PLASMA-EXCHANGE; ADULT PATIENTS; ECULIZUMAB; MANAGEMENT; GUIDELINES;
D O I
10.1097/XCE.0000000000000247
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Atypical hemolytic uremic syndrome (aHUS), a challenging disorder, commonly caused by inherited defects or regulatory processes of the complement alternative pathway. There are multiple causes, including pregnancy. Pregnancy provokes life-threatening episodes, preeclampsia, hemolysis elevated liver enzymes low platelets, microangiopathic hemolytic anemia (MAHA) and end-stage renal disease. Additionally, complement dysregulation and, with aHUS, affects fetal and maternal outcomes. Pregnancy-associated aHUS results in a poor prognosis with irreversible renal damage. Likewise, it is imperative to know that MAHA can provoke endothelial disruption, destruction of red cells and thrombocytopenia. We present a case of a young 18-year-old woman with MAHA and aHUS, requiring emergent cesarean section at 34 weeks of gestation and hemodialysis, secondary to complications from a recent pregnancy. Elevated blood pressure readings, rising creatinine levels, as well as her mother being on dialysis after pregnancy raised suspicion for thrombotic microangiopathy and aHUS. She was subsequently managed with plasma exchange, steroids, eculizumab and hemodialysis. Thus, plasma exchange should be initiated, with pending additional workup. Upon a definitive diagnosis of aHUS, eculizumab would be warranted to mitigate immune dysregulation. Understanding thrombotic microangiopathies diagnosis, and recognizing concomitant consequences, is vital. Having better insights into endothelial injuries can prevent unfortunate outcomes. Copyright (c) 2021 The Author(s). Published by Wolters Kluwer Health, Inc.
引用
收藏
页码:225 / 230
页数:6
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