ArticlePediatric nephrology (Berlin, Germany)2018
Extra-Renal manifestations of atypical hemolytic uremic syndrome in children.
Article in Pediatric nephrology (Berlin, Germany), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.
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Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 48 citations in OpenAlex.
- Extrarenal manifestations of atypical hemolytic uremic syndrome: a systematic review and meta-analysis.Pediatric research · 2025Pooled it
- Influenza A(H1N1) triggered atypical hemolytic uremic syndrome in a child with homozygous CD46 variant successfully treated with ravulizumab: a case report.CEN case reports · 2026Article
- Incidence and severity of extrarenal manifestations and outcomes in pediatric hemolytic uremic syndrome: a retrospective cohort study.Pediatric nephrology (Berlin, Germany) · 2026Article
- Updates in atypical hemolytic syndrome.Current opinion in pediatrics · 2026Review
- Demographics and baseline disease characteristics of UK patients within the global aHUS registry.BMC nephrology · 2025Article
- Neurological Manifestations of Hemolytic Uremic Syndrome: A Comprehensive Review.Brain sciences · 2025Review
- Potentially reversible severe cardiac involvement in thrombotic microangiopathies with malignant hypertension.Journal of nephrology · 2025Article
- Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies.Childhood kidney diseases · 2025Review
- Atypical Hemolytic Uremic Syndrome: A Review of Complement Dysregulation, Genetic Susceptibility and Multiorgan Involvement.Journal of clinical medicine · 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
- Unprecedented Haemorrhagic Stroke: A Rare Manifestation of Atypical Haemolytic Syndrome.Cureus · 2024Article
- Article
- Epidemiology, Management, and Outcome of Atypical Hemolytic Uremic Syndrome in an Omani Cohort.Oman medical journal · 2023Article
- Clinical features and management of atypical hemolytic uremic syndrome patient withFrontiers in pediatrics · 2023Review
- Genetic variation in genes of inborn errors of immunity in children with unexplained encephalitis.Genes and immunity · 2022Article
- Thrombotic microangiopathy following aortic surgery with hypothermic circulatory arrest: a single-centre experience of an underestimated cause of acute renal failure.Interactive cardiovascular and thoracic surgery · 2022Article
- Review
- Article
- Hemolytic Uremic Syndrome in Children.Turkish archives of pediatrics · 2021Article
- Acute ST-Segment Elevation Myocardial Infarction as Initial Presentation of Atypical Hemolytic-Uremic Syndrome.JACC. Case reports · 2021Article
Corrections and comments
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Authors and funding
11 authors at 7 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAtypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood.
methodsThis study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry. The demographics, clinical characteristics, genetic test results, all treatments, and renal/hematologic status of aHUS patients with extrarenal involvement were recorded.
resultsThe most common extrarenal manifestation was neurological system involvement (n = 46 [27.2%]), followed by gastrointestinal (n = 20 [11.8%]), cardiovascular (n = 12 [7%]), and respiratory (n = 12 [7%]) involvement. The patients with neurological involvement had a higher mortality rate and a lower estimated glomerular filtration rate (eGFR) than the other patients at last follow-up. Eculizumab (with or without plasma exchange/plasma infusion) treatment increased the renal and hematologic recovery rates.
conclusionsThe most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians.
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