Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review. Issue 4 (17th March 2021)

• Record Type: Journal Article
• Authors: Cadet, Bair; Meshoyrer, Daniel; Kim, Zae
• Is Part Of: Cardiovascular endocrinology & metabolism. Volume 10:Issue 4(2021)
• Page Start: 225
• Page End: 230
• Publication Date: 2021-03-17
• Subjects: atypical hemolytic uremic syndrome -- pregnancy -- end-stage renal disease; Cardiovascular system -- Diseases -- Periodicals; Endocrine glands -- Diseases -- Periodicals; Endocrinology -- Periodicals; Metabolism -- Disorders -- Periodicals; Diabetes -- Periodicals; 616.1
• DOI: 10.1097/XCE.0000000000000247
• Languages: English
• ISSNs: 2574-0954

Abstract

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.