CC BY-NC-ND 4.0 · AJP Rep 2021; 11(02): e95-e98
DOI: 10.1055/s-0041-1731057
Case Report

Neonatal Atypical Hemolytic Uremic Syndrome in the Eculizumab Era

Sara Madureira Gomes
1  Department of Pediatrics, Centro Hospitalar Universitário de São João, Porto, Portugal
,
Rita Pissarra Teixeira
1  Department of Pediatrics, Centro Hospitalar Universitário de São João, Porto, Portugal
,
Gustavo Rocha
2  Department of Neonatology, Centro Hospitalar Universitário de São João, Porto, Portugal
,
Paulo Soares
2  Department of Neonatology, Centro Hospitalar Universitário de São João, Porto, Portugal
,
Hercilia Guimaraes
3  Department of Pediatrics, Faculty of Medicine, University of Porto, Porto, Portugal
,
Paulo Santos
4  Department of Pediatric Cardiology, Centro Hospitalar Universitário de São João, Porto, Portugal
,
Joana Jardim
5  Pediatric Nephrology Unit, Department of Pediatrics, Centro Hospitalar Universitário de São João, Porto, Portugal
,
João Luís Barreira
5  Pediatric Nephrology Unit, Department of Pediatrics, Centro Hospitalar Universitário de São João, Porto, Portugal
,
Helena Pinto
5  Pediatric Nephrology Unit, Department of Pediatrics, Centro Hospitalar Universitário de São João, Porto, Portugal
› Author Affiliations

Abstract

The atypical hemolytic uremic syndrome (aHUS) in the newborn is a rare disease, with high morbidity. Eculizumab, considered a first-line drug in older children, is not approved in neonates and in children weighing less than 5 kg. We present a 5-day-old female newborn, born at 36 weeks' twin gestation, by emergency cesarean section due to cord prolapse, with birth weight of 2,035 g and Apgar score of 7/7/7, who develops microangiopathic hemolytic anemia, thrombocytopenia, and progressive acute renal failure. In day 5, after diagnosis of aHUS, a daily infusion of fresh frozen plasma begins, with improvement of thrombocytopenia and very slight improvement in renal function. The etiologic study (congenital infection, Shiga toxin, ADAMTS13 activity, directed metabolic study) was normal. C3c was slightly decreased. On day 16 for maintenance of anemia and severe renal failure, she started 300 mg/dose eculizumab. Anemia resolves in 10 weeks and creatinine has normal values after 13 weeks of treatment. The genetic study was normal. In this case, eculizumab is effective in controlling microangiopathy and in the recovery of renal function. Diagnosis of neonatal aHUS can be challenging because of phenotypic heterogeneity and potential overlap with other manifestations that may confound it, such as perinatal asphyxia or sepsis/disseminated intravascular coagulation.



Publication History

Received: 15 February 2021

Accepted: 25 March 2021

Publication Date:
23 June 2021 (online)

© 2021. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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