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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...

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Detalles Bibliográficos
Autores principales: Cadet, Bair, Meshoyrer, Daniel, Kim, Zae
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8575437/
https://www.ncbi.nlm.nih.gov/pubmed/34765894
http://dx.doi.org/10.1097/XCE.0000000000000247
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author Cadet, Bair
Meshoyrer, Daniel
Kim, Zae
author_facet Cadet, Bair
Meshoyrer, Daniel
Kim, Zae
author_sort Cadet, Bair
collection PubMed
description 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.
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spelling pubmed-85754372021-11-10 Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review Cadet, Bair Meshoyrer, Daniel Kim, Zae Cardiovasc Endocrinol Metab Case Report 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. Wolters Kluwer Health 2021-03-17 /pmc/articles/PMC8575437/ /pubmed/34765894 http://dx.doi.org/10.1097/XCE.0000000000000247 Text en Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Case Report
Cadet, Bair
Meshoyrer, Daniel
Kim, Zae
Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review
title Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review
title_full Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review
title_fullStr Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review
title_full_unstemmed Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review
title_short Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review
title_sort atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8575437/
https://www.ncbi.nlm.nih.gov/pubmed/34765894
http://dx.doi.org/10.1097/XCE.0000000000000247
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