ArticlePediatric nephrology (Berlin, Germany)2015
Eculizumab in the treatment of atypical hemolytic uremic syndrome in an infant leads to cessation of peritoneal dialysis and improvement of severe hypertension.
Article in Pediatric nephrology (Berlin, Germany), 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 18 citations in OpenAlex.
- Advances in Research on the Release of von Willebrand Factor from Endothelial Cells through the Membrane Attack Complex C5b-9 in Sepsis.Journal of inflammation research · 2025Review
- C3 molecular structural and histopathological analyses in a pediatric case of atypical hemolytic uremic syndrome with life-threatening gastrointestinal bleeding-a case report.Frontiers in pediatrics · 2025Article
- Protein-losing enteropathy as a new phenotype in atypical hemolytic uremic syndrome caused by CD46 gene mutation.Pediatric nephrology (Berlin, Germany) · 2024Review
- Prophylactic amoxicillin for the prevention of meningococcal infection in infants with atypical hemolytic uremic syndrome under treatment with eculizumab: a report of two cases.CEN case reports · 2020Article
- Is eculizumab indicated in patients with atypical hemolytic uremic syndrome already on prolonged dialysis? A case report and review of the literature.Pediatric nephrology (Berlin, Germany) · 2019Review
- Eculizumab Safety: Five-Year Experience From the Global Atypical Hemolytic Uremic Syndrome Registry.Kidney international reports · 2019Article
- Hypertension: a new treatment for an old disease? Targeting the immune system.British journal of pharmacology · 2019Review
- Pathogenesis of Atypical Hemolytic Uremic Syndrome.Journal of atherosclerosis and thrombosis · 2019Review
- Clinical characteristics and genetic backgrounds of Japanese patients with atypical hemolytic uremic syndrome.Clinical and experimental nephrology · 2018Article
- Hypertensive Crisis and Refractory Hypertension Caused by Atypical Hemolytic Uremic Syndrome and Effect of Eculizumab.Acta Cardiologica Sinica · 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
- Critical appraisal of eculizumab for atypical hemolytic uremic syndrome.Journal of blood medicine · 2016Review
- Neonatal atypical hemolytic uremic syndrome from a factor H mutation treated with eculizumab.Clinical nephrology · 2015Article
- Timing of eculizumab therapy for C3 glomerulonephritis.Clinical kidney journal · 2015Article
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Authors and funding
12 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSevere hypertension (HTN) and acute kidney injury frequently associated with atypical hemolytic uremic syndrome (aHUS) were refractory to various therapies in the pre-eculizumab era. Here we report the case of a 4-month-old boy who developed aHUS presenting with undetectable C3 protein, no predisposing mutations in complement factors, and no antibodies against factor H.
methodsRepeated plasma infusions and nine sessions of plasmapheresis were ineffective. The patient initially required continuous hemodiafiltration and thereafter peritoneal dialysis. Despite vigorous antihypertensive treatment and improved fluid overload with dialysis, HTN persisted. His low C3 level (<20 mg/dl) suggested unrestricted complement activation. Therefore, based on the suspicion of unrestricted complement cascade in the pathogenesis, treatment with eculizumab, a human anti-C5 monoclonal antibody, was initiated with the aim of controlling disease activity.
resultsEculizumab therapy resulted in the control of severe HTN and cessation of peritoneal dialysis.
conclusionsThis infant with HTN and acute kidney injury associated with aHUS was treated successfully with eculizumab.
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