ArticlePediatric nephrology (Berlin, Germany)2013
Eculizumab therapy for atypical haemolytic uraemic syndrome due to a gain-of-function mutation of complement factor B.
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 13 papers.
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Who cites it
13 citing papers in PubMed, 22 citations in OpenAlex.
- The Inhibitory Effects of a Factor B-Binding DNA Aptamer Family Supersede the Gain of Function of Factor B Variants Associated with Atypical Hemolytic Uremic Syndrome.Journal of immunology (Baltimore, Md. : 1950) · 2024Article
- Clinical Relapses of Atypical HUS on Eculizumab: Clinical Gap for Monitoring and Individualised Therapy.Case reports in nephrology · 2018Article
- Diabetic ketoacidosis presenting with atypical hemolytic uremic syndrome associated with a variant of complement factor B in an adult: a case report.Journal of medical case reports · 2016Article
- An international consensus approach to the management of atypical hemolytic uremic syndrome in children.Pediatric nephrology (Berlin, Germany) · 2016Review
- 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
- Advances and challenges in the management of complement-mediated thrombotic microangiopathies.Therapeutic advances in hematology · 2015Review
- A complicated case of atypical hemolytic uremic syndrome with frequent relapses under eculizumab.Pediatric nephrology (Berlin, Germany) · 2015Article
- Complement factor B mutations in atypical hemolytic uremic syndrome-disease-relevant or benign?Journal of the American Society of Nephrology : JASN · 2014Article
- Hemolytic uremic syndrome.Seminars in immunopathology · 2014Review
- Eculizumab in atypical haemolytic uraemic syndrome with severe cardiac and neurological involvement.Pediatric nephrology (Berlin, Germany) · 2014Article
- Eculizumab therapy leads to rapid resolution of thrombocytopenia in atypical hemolytic uremic syndrome.Advances in hematology · 2014Article
- Complement therapy in atypical haemolytic uraemic syndrome (aHUS).Molecular immunology · 2013Review
Corrections and comments
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Authors and funding
6 authors at 5 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAtypical haemolytic uraemic syndrome (aHUS) is caused by dysregulated complement activation. A humanised anti-C5 monoclonal antibody has recently become available for treatment of this condition CASE-DIAGNOSIS/TREATMENT: We present the first description of an infant with an activating mutation of complement factor B successfully treated with eculizumab. On standard doses she had evidence of ongoing C5 cleavage despite a good clinical response.
conclusionsEculizumab is effective therapy for aHUS associated with factor B mutations, but recommended doses may not be adequate for all patients.
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