ArticlePediatric nephrology (Berlin, Germany)2014
Macrovascular involvement in a child with atypical hemolytic uremic syndrome.
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 16 papers.
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Who cites it
16 citing papers in PubMed, 20 citations in OpenAlex.
- Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies.Kidney international · 2015Trial
- Multisystem involvement with ischemic complications in a child with STEC-HUS: a case of gangrene.Pediatric nephrology (Berlin, Germany) · 2026Article
- Atypical Hemolytic-Uremic Syndrome: Genetic Basis, Clinical Manifestations, and a Multidisciplinary Approach to Management.Journal of multidisciplinary healthcare · 2023Review
- Identification of discriminative gene-level and protein-level features associated with pathogenic gain-of-function and loss-of-function variants.American journal of human genetics · 2021Article
- Article
- Eculizumab in paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome: 10-year pharmacovigilance analysis.British journal of haematology · 2019Article
- Atypical hemolytic uremic syndrome and eculizumab therapy in children.Korean journal of pediatrics · 2018Review
- Complement factor B mutation-associated aHUS and myocardial infarction.BMJ case reports · 2017Article
- Rapid conditional targeted ablation model for hemolytic anemia in the rat.Physiological genomics · 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
- Article
- New functional and structural insights from updated mutational databases for complement factor H, Factor I, membrane cofactor protein and C3.Bioscience reports · 2014Article
- Current treatment of atypical hemolytic uremic syndrome.Intractable & rare diseases research · 2014Review
- Cardiovascular complications in atypical haemolytic uraemic syndrome.Nature reviews. Nephrology · 2014Review
- Extra-renal manifestations of complement-mediated thrombotic microangiopathies.Frontiers in pediatrics · 2014Review
Corrections and comments
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Authors and funding
5 authors at 5 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAtypical hemolytic uremic syndrome (aHUS) is a disorder of the complement system which leads to thrombotic microangiopathy. It is caused by either acquired or hereditary defects in the activation or regulation of the alternative complement pathway and is therefore considered to be a disease of local complement dysregulation in microvasculature with predominantly renal involvement. However, extrarenal manifestations are observed in approximately one-fifth of aHUS patients, with the myocardium and central nervous system (CNS) being involved most often. Additionally, there have been a few reports of aHUS with cerebral artery stenoses or periphereal gangrene, suggesting the possibility of 'macrovascular' involvement in aHUS. CASE-DIAGNOSIS/TREATMENT: We present a child with early onset aHUS and a C3 gain-of-function mutation who developed cerebral artery stenoses, leading ultimately to death due to a massive stroke 9 days after successful renal transplantation under prophylactic eculizumab treatment. Similar cases described in the literature are also briefly summarized.
conclusionsThe disease course in our patient with aHUS confirms that dysregulated complement activation can induce arterial steno-occlusive lesions in the absence of acute episodes of HUS. Further studies are required to document the frequency of such macrovascular complications and the role of eculizumab treatment in preventing their development and progression.
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