ArticlePediatric nephrology (Berlin, Germany)2013
Neonatal onset atypical hemolytic uremic syndrome successfully treated with eculizumab.
Article in Pediatric nephrology (Berlin, Germany), 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 41 citations in OpenAlex.
- Hemolytic Uremic Syndrome in Children.Turkish archives of pediatrics · 2021Article
- Neonatal Atypical Hemolytic Uremic Syndrome in the Eculizumab Era.AJP reports · 2021Article
- Case Report: Neonatal Unexplained HUS Treated With Complement Inhibitor Eculizumab.Frontiers in pediatrics · 2020Article
- Renal failure, respiratory distress, and an atypical purpuric rash in a full-term infant with omphalocele and hypospadias: Answers.Pediatric nephrology (Berlin, Germany) · 2019Article
- Clinical Relapses of Atypical HUS on Eculizumab: Clinical Gap for Monitoring and Individualised Therapy.Case reports in nephrology · 2018Article
- Atypical hemolytic uremic syndrome in the setting of complement-amplifying conditions: case reports and a review of the evidence for treatment with eculizumab.Journal of nephrology · 2017Review
- Turkish pediatric atypical hemolytic uremic syndrome registry: initial analysis of 146 patients.BMC nephrology · 2017Article
- First-Line, Early and Long-Term Eculizumab Therapy in Atypical Hemolytic Uremic Syndrome: A Case Series in Pediatric Patients.Paediatric drugs · 2016Article
- Proteinuria and hematuria in the neonate.Current opinion in pediatrics · 2016Review
- Efficacy and safety of eculizumab in childhood atypical hemolytic uremic syndrome in Japan.Clinical and experimental nephrology · 2016Article
- Critical appraisal of eculizumab for atypical hemolytic uremic syndrome.Journal of blood medicine · 2016Review
- An international consensus approach to the management of atypical hemolytic uremic syndrome in children.Pediatric nephrology (Berlin, Germany) · 2016Review
- Exchange transfusion for neonate with haemolytic uremic syndrome.SpringerPlus · 2016Article
- Neonatal atypical hemolytic uremic syndrome from a factor H mutation treated with eculizumab.Clinical nephrology · 2015Article
- First-line therapy in atypical hemolytic uremic syndrome: consideration on infants with a poor prognosis.Italian journal of pediatrics · 2014Article
- Eculizumab in neonatal hemolytic uremic syndrome with homozygous factor H deficiency.Pediatric nephrology (Berlin, Germany) · 2014Article
- Eculizumab in atypical haemolytic uraemic syndrome with severe cardiac and neurological involvement.Pediatric nephrology (Berlin, Germany) · 2014Article
- Eculizumab therapy for atypical haemolytic uraemic syndrome due to a gain-of-function mutation of complement factor B.Pediatric nephrology (Berlin, Germany) · 2013Article
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Authors and funding
7 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAtypical hemolytic uremic syndrome (aHUS) is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Neonatal cases are extremely uncommon. Plasma therapy is the first choice therapy in patients with aHUS based on the belief of an underlying complement dysregulation. Alternatively, eculizumab, which targets complement 5, is used to block complement activation. CASE-DIAGNOSIS/TREATMENT: Sudden onset macroscopic hematuria, hypertension, and bruises over the entire body were noted in a 5 day-old newborn. Investigations revealed hemolytic anemia, thrombocytopenia, renal impairment, and a low serum C3, leading to the diagnosis of aHUS. Fresh frozen plasma (FFP) infusions and peritoneal dialysis for acute kidney injury were initiated. This approach yielded full renal and hematological remission. The patient was discharged with FFP infusions, but subsequently developed three life-threatening disease recurrences at 1, 3, and 6 months of age. The last relapse presented with uncontrolled hypertension and impaired renal function while the patient was receiving FFP infusions. After the first dose of eculizumab, his renal and hematological parameters returned to normal and his blood pressure normalized. Genetic screening of the CFH gene revealed a novel homozygous p. Tyr1177Cys mutation.
conclusionEculizumab can be considered as an alternative to plasma therapy in the treatment of specific patients with aHUS, even in infants.
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