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非典型溶血性尿毒症综合征:妊娠导致终身透析的病例报告及文献综述

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

作者信息

Cadet Bair, Meshoyrer Daniel, Kim Zae

机构信息

Department of Internal Medicine, Division of Nephrology.

Department of Family Medicine, Nassau University Medical Center, East Meadow, New York, USA.

出版信息

Cardiovasc Endocrinol Metab. 2021 Mar 17;10(4):225-230. doi: 10.1097/XCE.0000000000000247. eCollection 2021 Dec.

Abstract

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.

摘要

非典型溶血尿毒综合征(aHUS)是一种具有挑战性的疾病,通常由补体替代途径的遗传缺陷或调节过程引起。其病因多样,包括妊娠。妊娠会引发危及生命的发作、先兆子痫、溶血、肝酶升高、血小板减少、微血管病性溶血性贫血(MAHA)和终末期肾病。此外,补体失调与aHUS一起,会影响胎儿和母亲的结局。妊娠相关的aHUS预后不良,会导致不可逆的肾损伤。同样,必须知道MAHA可引发内皮细胞破坏、红细胞破坏和血小板减少。我们报告一例18岁年轻女性,患有MAHA和aHUS,因近期妊娠并发症,在妊娠34周时需要紧急剖宫产和血液透析。血压读数升高、肌酐水平上升,以及她的母亲在妊娠后接受透析,引发了对血栓性微血管病和aHUS的怀疑。她随后接受了血浆置换、类固醇、依库珠单抗和血液透析治疗。因此,应在进一步检查之前启动血浆置换。一旦确诊为aHUS,依库珠单抗将有助于减轻免疫失调。了解血栓性微血管病的诊断并认识到其伴随的后果至关重要。更好地了解内皮损伤可预防不良后果。

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