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Neonatal Atypical Hemolytic Uremic Syndrome in the Eculizumab Era

Authors
  • Gomes, Sara Madureira1
  • Teixeira, Rita Pissarra1
  • Rocha, Gustavo2
  • Soares, Paulo2
  • Guimaraes, Hercilia3
  • Santos, Paulo4
  • Jardim, Joana5
  • Barreira, João Luís5
  • Pinto, Helena5
  • 1 Department of Pediatrics, Centro Hospitalar Universitário de São João, Porto, Portugal
  • 2 Department of Neonatology, Centro Hospitalar Universitário de São João, Porto, Portugal
  • 3 Department of Pediatrics, Faculty of Medicine, University of Porto, Porto, Portugal
  • 4 Department of Pediatric Cardiology, Centro Hospitalar Universitário de São João, Porto, Portugal
  • 5 Pediatric Nephrology Unit, Department of Pediatrics, Centro Hospitalar Universitário de São João, Porto, Portugal
Type
Published Article
Journal
AJP Reports
Publisher
Thieme Medical Publishers, Inc.
Publication Date
Apr 01, 2021
Volume
11
Issue
2
Identifiers
DOI: 10.1055/s-0041-1731057
PMID: 34178424
PMCID: PMC8221835
Source
PubMed Central
Keywords
Disciplines
  • Case Report
License
Unknown

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.

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