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Case Reports
. 2016 Dec 20;6(3):143-148.
doi: 10.1159/000454946. eCollection 2016 Sep-Dec.

Pregnant Woman with Atypical Hemolytic Uremic Syndrome Delivered a Healthy Newborn under Eculizumab Treatment

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Case Reports

Pregnant Woman with Atypical Hemolytic Uremic Syndrome Delivered a Healthy Newborn under Eculizumab Treatment

Erol Demir et al. Case Rep Nephrol Dial. .

Abstract

Pregnancy-associated thrombotic microangiopathy is a very rare condition; however, it significantly increases fetal or maternal morbidity and mortality. Pregnancy may trigger atypical hemolytic uremic syndrome (aHUS) or thrombotic thrombocytopenic purpura. The risk for pregnancy-associated aHUS is highest during the second pregnancy. The outcome is usually poor with 50-60% mortality; renal dysfunction and hypertension are the rule in those who survive the acute episode. After the development of complement regulation mechanisms and aHUS pathogenesis, eculizumab has been widely used as a first-line treatment in aHUS. Eculizumab has been produced to minimize immunogenicity and Fc-mediated functions, including recruitment of inflammatory cells and complement activation, and using eculizumab in first-line treatment improves kidney function. Recent studies showed that early diagnosis and rapid use of eculizumab in first-line treatment improve outcomes. We demonstrate a case with pregnancy-triggered aHUS occurring in the second trimester, who was successfully treated and delivered a healthy baby under eculizumab treatment.

Keywords: Eculizumab; Pregnancy-associated atypical hemolytic uremic syndrome; Pregnancy-associated thrombotic microangiopathy.

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Figures

Fig. 1.
Fig. 1.
a Thrombi within glomerular capillary lumina (HE, ×100). bd Endothelial swelling, mesangiolysis, and glomerular basement membrane duplication (b, c ×200; d PAMS, ×200).

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