ArticlePediatric nephrology (Berlin, Germany)2014
Eculizumab in atypical haemolytic uraemic syndrome with severe cardiac and neurological involvement.
Article in Pediatric nephrology (Berlin, Germany), 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed.
- Early recognition of atypical hemolytic uremic syndrome to prevent irreversible kidney injury: cardiac failure and refractory hypertension as critical clues in young patients.CEN case reports · 2026Article
- Favorable Outcome With Eculizumab in Hemolytic Uremic Syndrome Presenting With Severe Neurological Complications: A Case Report.Case reports in nephrology · 2026Article
- [Ultra-early administration of eculizumab in a child with atypical hemolytic uremic syndrome: a case report].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2025Article
- Neurological involvement in hematopoietic stem cell transplantation-associated thrombotic microangiopathy.Annals of hematology · 2024Review
- Cerebral vascular injury in transplant-associated thrombotic microangiopathy.Blood advances · 2022Article
- Review
- Familial Atypical Hemolytic Uremic Syndrome with Positive p.S1191L (c.3572C>T) Mutation on theBalkan journal of medical genetics : BJMG · 2021Article
- Article
- Atypical haemolytic uraemic syndrome: a case report of a rare cause of reversible cardiomyopathy.European heart journal. Case reports · 2020Article
- Extra-renal manifestations of atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2019Review
- Hemolytic uremic syndrome in a developing country: Consensus guidelines.Pediatric nephrology (Berlin, Germany) · 2019Article
- Alteration of glycosylation in serum proteins: a new potential indicator to distinguish non-diabetic renal diseases from diabetic nephropathy.RSC advances · 2018Article
- An adult case of atypical hemolytic uremic syndrome presented with posterior reversible encephalopathy syndrome: Successful response to late-onset eculizumab treatment.Hematology reports · 2018Article
- Extra-Renal manifestations of atypical hemolytic uremic syndrome in children.Pediatric nephrology (Berlin, Germany) · 2018Article
- Complement factor B mutation-associated aHUS and myocardial infarction.BMJ case reports · 2017Article
- 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
- Remission of aHUS neurological damage with eculizumab.Clinical kidney journal · 2015Article
- Case report of atypical hemolytic uremic syndrome with retinal arterial and venous occlusion treated with eculizumab.International medical case reports journal · 2015Article
- Extra-renal manifestations of complement-mediated thrombotic microangiopathies.Frontiers in pediatrics · 2014Review
Corrections and comments
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAtypical haemolytic uraemic syndrome (aHUS) is a rare disorder usually caused by dysregulation of the alternative complement pathway. Uncontrolled complement activation results in systemic complement-mediated thrombotic microangiopathy (TMA) and subsequent multi-organ damage. The two most common extrarenal complications comprise neurological and cardiovascular involvement. Eculizumab, a humanised anti-C5 monoclonal antibody, has recently been introduced as a therapy for this condition. CASE-DIAGNOSIS/TREATMENT: A 19-month-old child suffering from aHUS with severe neurological involvement, dilated cardiomyopathy and renal impairment requiring dialysis received eculizumab as first-line treatment, initiated within 12 h of admission, resulting in significant improvements in her neurological state and normalisation of cardiac and renal function. These positive outcomes have been sustained with fortnightly eculizumab therapy (at the time of writing, on-going for 1 year). No further complications of TMA have occurred.
conclusionSevere cardiac involvement in a child with aHUS is an important indication for prompt, first-line treatment with eculizumab, resulting in rapid normalisation of cardiac function.
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