非典型溶血性尿毒症综合征:当怀孕导致终身透析:1例报告和文献复习。

IF 1.3 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS
Cardiovascular Endocrinology & Metabolism Pub Date : 2021-03-17 eCollection Date: 2021-12-01 DOI:10.1097/XCE.0000000000000247
Bair Cadet, Daniel Meshoyrer, Zae Kim
{"title":"非典型溶血性尿毒症综合征:当怀孕导致终身透析:1例报告和文献复习。","authors":"Bair Cadet,&nbsp;Daniel Meshoyrer,&nbsp;Zae Kim","doi":"10.1097/XCE.0000000000000247","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":43231,"journal":{"name":"Cardiovascular Endocrinology & Metabolism","volume":null,"pages":null},"PeriodicalIF":1.3000,"publicationDate":"2021-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ec/86/xce-10-225.PMC8575437.pdf","citationCount":"1","resultStr":"{\"title\":\"Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review.\",\"authors\":\"Bair Cadet,&nbsp;Daniel Meshoyrer,&nbsp;Zae Kim\",\"doi\":\"10.1097/XCE.0000000000000247\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":43231,\"journal\":{\"name\":\"Cardiovascular Endocrinology & Metabolism\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.3000,\"publicationDate\":\"2021-03-17\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ec/86/xce-10-225.PMC8575437.pdf\",\"citationCount\":\"1\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Cardiovascular Endocrinology & Metabolism\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1097/XCE.0000000000000247\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2021/12/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"CARDIAC & CARDIOVASCULAR SYSTEMS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Cardiovascular Endocrinology & Metabolism","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1097/XCE.0000000000000247","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2021/12/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
引用次数: 1

摘要

非典型溶血性尿毒症综合征(aHUS)是一种具有挑战性的疾病,通常由遗传缺陷或补体替代途径的调节过程引起。有多种原因,包括怀孕。妊娠可引发危及生命的发作、先兆子痫、溶血、肝酶升高、血小板降低、微血管病溶血性贫血(MAHA)和终末期肾病。此外,补体失调和与aHUS,影响胎儿和母亲的结局。妊娠相关性aHUS预后不良,伴有不可逆的肾损害。同样,必须知道MAHA可引起内皮破坏、红细胞破坏和血小板减少症。我们报告了一例年轻的18岁女性MAHA和aHUS,在妊娠34周时需要紧急剖宫产和血液透析,继发于近期妊娠的并发症。血压读数升高,肌酐水平升高,以及她的母亲在怀孕后进行透析,这些都增加了对血栓性微血管病和aHUS的怀疑。随后,她接受了血浆置换、类固醇、eculizumab和血液透析治疗。因此,应开始血浆置换,等待额外的检查。在aHUS的明确诊断,eculizumab将保证减轻免疫失调。了解血栓性微血管病变的诊断,并认识到随之而来的后果,是至关重要的。对内皮损伤有更好的了解可以防止不幸的结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review.

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.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Cardiovascular Endocrinology & Metabolism
Cardiovascular Endocrinology & Metabolism CARDIAC & CARDIOVASCULAR SYSTEMS-
CiteScore
5.60
自引率
0.00%
发文量
24
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信