ArticleClinical kidney journal2019
Atypical hemolytic uremic syndrome: a syndrome in need of clarity.
Article in Clinical kidney journal, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed.
- Comprehensive gene profiling by Next-Generation sequencing in a cohort of Egyptian pediatric Atypical HUS.Journal, genetic engineering & biotechnology · 2026Article
- Management of Atypical Haemolytic Uraemic Syndrome With Triggers: Diagnostic and Treatment Algorithms From an Asia-Pacific Perspective.Nephrology (Carlton, Vic.) · 2025Review
- 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
- Systematic Review of Individual Patient Data COVID-19 Infection and Vaccination-Associated Thrombotic Microangiopathy.Kidney international reports · 2024Article
- The Role of Complement in HSCT-TMA: Basic Science to Clinical Practice.Advances in therapy · 2022Review
- Complement Factor I Variants in Complement-Mediated Renal Diseases.Frontiers in immunology · 2022Article
- Postoperative Atypical Hemolytic Uremic Syndrome in a 67-Year-Old Woman Associated with Hemolytic Anemia, Thrombocytopenia, and Acute Renal Failure.The American journal of case reports · 2020Article
- Article
- Treatment of Rare Inflammatory Kidney Diseases: Drugs Targeting the Terminal Complement Pathway.Frontiers in immunology · 2020Review
- Epidemiology of Atypical Hemolytic Uremic Syndrome: A Systematic Literature Review.Clinical epidemiology · 2020Review
- Diagnostic Testing for Differential Diagnosis in Thrombotic MicroangiopathiesTurkish journal of haematology : official journal of Turkish Society of Haematology · 2019Review
- An updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy.Clinical kidney journal · 2019Article
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Authors and funding
1 author.
Funding
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Abstract
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) originally understood to be limited to renal and hematopoietic involvement. Whereas aberrations in complement regulatory proteins (CRPs), C3 or complement factor B (CFB) are detected in ∼60% of patients, a complement-derived pathogenesis that reflects dysregulation of the alternative pathway (AP) of complement activation is present in ∼90% of patients. aHUS remains a diagnosis of exclusion. The discovery of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) and its utility in the diagnosis of thrombotic thrombocytopenic purpura (TTP) has resulted in the appreciation that cases of aHUS have been inappropriately diagnosed as TTP. Thus there has been an evolving appreciation of clinical manifestations of aHUS that renders the appellation aHUS misleading. This article will review the pathogenesis and the evolving clinical presentations of aHUS, present a hypothesis that there can be a phenotypic expression of aHUS due to a complement storm in a disorder where direct endothelial damage occurs and discuss future areas of research to more clearly define the clinical spectrum and management of aHUS.
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