{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,8,24]],"date-time":"2023-08-24T01:59:42Z","timestamp":1692842382384},"reference-count":22,"publisher":"Georg Thieme Verlag KG","issue":"02","license":[{"start":{"date-parts":[[2021,6,23]],"date-time":"2021-06-23T00:00:00Z","timestamp":1624406400000},"content-version":"vor","delay-in-days":83,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["AJP Rep"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>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\u2009kg. 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\u2009g 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\u2009mg\/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.<\/jats:p>","DOI":"10.1055\/s-0041-1731057","type":"journal-article","created":{"date-parts":[[2021,6,23]],"date-time":"2021-06-23T22:56:14Z","timestamp":1624488974000},"page":"e95-e98","source":"Crossref","is-referenced-by-count":1,"title":["Neonatal Atypical Hemolytic Uremic Syndrome in the Eculizumab Era"],"prefix":"10.1055","volume":"11","author":[{"given":"Sara Madureira","family":"Gomes","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o, Porto, Portugal"}]},{"given":"Rita Pissarra","family":"Teixeira","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o, Porto, Portugal"}]},{"given":"Gustavo","family":"Rocha","sequence":"additional","affiliation":[{"name":"Department of Neonatology, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o, Porto, Portugal"}]},{"given":"Paulo","family":"Soares","sequence":"additional","affiliation":[{"name":"Department of Neonatology, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o, Porto, Portugal"}]},{"given":"Hercilia","family":"Guimaraes","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine, University of Porto, Porto, Portugal"}]},{"given":"Paulo","family":"Santos","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o, Porto, Portugal"}]},{"given":"Joana","family":"Jardim","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Unit, Department of Pediatrics, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o, Porto, Portugal"}]},{"given":"Jo\u00e3o Lu\u00eds","family":"Barreira","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Unit, Department of Pediatrics, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o, Porto, Portugal"}]},{"given":"Helena","family":"Pinto","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Unit, Department of Pediatrics, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o, Porto, Portugal"}]}],"member":"194","published-online":{"date-parts":[[2021,6,23]]},"reference":[{"issue":"03","key":"ref1","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1136\/archdischild-2016-311377","article-title":"Treatment and management of children with haemolytic uraemic syndrome","volume":"103","author":"P R Walsh","year":"2018","journal-title":"Arch Dis Child"},{"issue":"03","key":"ref2","first-page":"1","article-title":"Portuguese consensus document statement in diagnostic and management of atypical hemolytic uremic 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