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Consensus regarding diagnosis and management of atypical hemolytic uremic syndrome.关于非典型溶血性尿毒症综合征的诊断和治疗的共识。
Korean J Intern Med. 2020 Jan;35(1):25-40. doi: 10.3904/kjim.2019.388. Epub 2020 Jan 2.
2
Diagnostic and Risk Factors for Complement Defects in Hypertensive Emergency and Thrombotic Microangiopathy.高血压急症和血栓性微血管病中补体缺陷的诊断和危险因素。
Hypertension. 2020 Feb;75(2):422-430. doi: 10.1161/HYPERTENSIONAHA.119.13714. Epub 2019 Dec 23.
3
Thrombomodulin and Endothelial Dysfunction: A Disease-Modifier Shared between Malignant Hypertension and Atypical Hemolytic Uremic Syndrome.血栓调节蛋白与血管内皮功能障碍:恶性高血压与非典型溶血尿毒综合征之间的共同疾病修饰因子。
Nephron. 2018;140(1):63-73. doi: 10.1159/000490201. Epub 2018 Jun 25.
4
Thrombotic microangiopathies of pregnancy: Differential diagnosis.妊娠期血栓性微血管病:鉴别诊断。
Pregnancy Hypertens. 2018 Apr;12:29-34. doi: 10.1016/j.preghy.2018.02.007. Epub 2018 Feb 16.
5
Microangiopathies in pregnancy.妊娠期微血管病
BMJ Case Rep. 2018 Jan 17;2018:bcr-2017-221648. doi: 10.1136/bcr-2017-221648.
6
Maternal and Fetal Outcomes of Pregnancies in Women with Atypical Hemolytic Uremic Syndrome.妊娠合并非典型溶血尿毒综合征患者的母婴结局。
J Am Soc Nephrol. 2018 Mar;29(3):1020-1029. doi: 10.1681/ASN.2016090995. Epub 2017 Dec 27.
7
Thrombotic Microangiopathies (TTP, HUS, HELLP).血栓性微血管病(血栓性血小板减少性紫癜、溶血尿毒综合征、伴有溶血、肝酶升高和血小板减少的子痫前期)
Hematol Oncol Clin North Am. 2017 Dec;31(6):1081-1103. doi: 10.1016/j.hoc.2017.08.010.
8
Belgian consensus statement on the diagnosis and management of patients with atypical hemolytic uremic syndrome.比利时关于非典型溶血尿毒症综合征患者诊断与管理的共识声明。
Acta Clin Belg. 2018 Feb;73(1):80-89. doi: 10.1080/17843286.2017.1345185. Epub 2017 Oct 23.
9
Clinical dissection of thrombotic microangiopathy.血栓性微血管病的临床剖析
Ann Hematol. 2017 Oct;96(10):1715-1726. doi: 10.1007/s00277-017-3063-1. Epub 2017 Jul 27.
10
HUS and atypical HUS.溶血尿毒综合征和非典型溶血尿毒综合征。
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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.

DOI:10.1097/XCE.0000000000000247
PMID:34765894
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8575437/
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。