ReviewIntractable & rare diseases research2014
Current treatment of atypical hemolytic uremic syndrome.
Review in Intractable & rare diseases research, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers, 3 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
33 citing papers in PubMed, 3 syntheses or guidelines pooled it, 68 citations in OpenAlex.
- Targeting the Roots of Kidney Disease: Systematic Review of the Therapies Targeting the Complement System.Medicina (Kaunas, Lithuania) · 2025Pooled it
- Interventions for atypical haemolytic uraemic syndrome.The Cochrane database of systematic reviews · 2021Pooled it
- A systematic review of the role of eculizumab in systemic lupus erythematosus-associated thrombotic microangiopathy.BMC nephrology · 2020Pooled it
- Incidence and severity of extrarenal manifestations and outcomes in pediatric hemolytic uremic syndrome: a retrospective cohort study.Pediatric nephrology (Berlin, Germany) · 2026Article
- A Unique Case of Atypical Hemolytic Uremic Syndrome Secondary to Alcohol-Induced Acute Pancreatitis.Cureus · 2026Article
- Cholecystectomy-induced thrombotic microangiopathy (TMA) in a postpartum patient successfully treated with eculizumab: a case report.Journal of medical case reports · 2024Article
- Unusual Presentation of Aggressive Atypical Hemolytic Uremic Syndrome With Brugada Syndrome.Cureus · 2024Article
- Immunogenicity of Recombinant Adeno-Associated Virus (AAV) Vectors for Gene Transfer.BioDrugs : clinical immunotherapeutics, biopharmaceuticals and gene therapy · 2023Review
- Application of a scoring system in Japanese patients diagnosed with atypical hemolytic uremic syndrome to assess the relationship between the score and clinical responses to eculizumab.Thrombosis journal · 2023Article
- Article
- C3 glomerulopathy and atypical hemolytic uremic syndrome: an updated review of the literature on alternative complement pathway disorders.International urology and nephrology · 2021Review
- Atypical hemolytic uremic syndrome due to DGKE mutation and response to eculizumab: lessons for the clinical nephrologist.Journal of nephrology · 2021Article
- Case Report: Acute Thrombotic Microangiopathy in a Patient with STING-Associated Vasculopathy with Onset in Infancy (SAVI).Journal of clinical immunology · 2020Article
- Gastrointestinal complications after kidney transplantation.World journal of gastroenterology · 2020Review
- New insights into the pathogenesis of Streptococcus pneumoniae-associated hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2020Review
- Epidemiology of Atypical Hemolytic Uremic Syndrome: A Systematic Literature Review.Clinical epidemiology · 2020Review
- Eculizumab Safety: Five-Year Experience From the Global Atypical Hemolytic Uremic Syndrome Registry.Kidney international reports · 2019Article
- Differential contribution of C5aR and C5b-9 pathways to renal thrombic microangiopathy and macrovascular thrombosis in mice carrying an atypical hemolytic syndrome-related factor H mutation.Kidney international · 2019Article
- Sepsis and septic shock: endothelial molecular pathogenesis associated with vascular microthrombotic disease.Thrombosis journal · 2019Review
- Targeting properdin in the treatment of atypical haemolytic uraemic syndrome: better than eculizumab?Annals of translational medicine · 2018Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Tremendous advances have been made in understanding the pathogenesis of atypical Hemolytic Uremic Syndrome (aHUS), an extremely rare disease. Insights into the molecular biology of aHUS resulted in rapid advances in treatment with eculizumab (Soliris(®), Alexion Pharmaceuticals Inc.). Historically, aHUS was associated with very high rates of mortality and morbidity. Prior therapies included plasma therapy and/or liver transplantation. Although often life saving, these were imperfect and had many complications. We review the conditions included under the rubric of aHUS: S. pneumoniae HUS (SpHUS), inborn errors of metabolism, and disorders of complement regulation, emphasizing their differences and similarities. We focus on the clinical features, diagnosis, and pathogenesis, and treatment of aHUS that results from mutations in genes encoding alternative complement regulators, SpHUS and HUS associated with inborn errors of metabolism. Mutations in complement genes, or antibodies to their protein products, result in unregulated activity of the alternate complement pathway, endothelial injury, and thrombotic microangiopathy (TMA). Eculizumab is a humanized monoclonal antibody that inhibits the production of the terminal complement components C5a and the membrane attack complex (C5b-9) by binding to complement protein C5a. This blocks the proinflammatory and cytolytic effects of terminal complement activation. Eculizumab use has been reported in many case reports, and retrospective and prospective clinical trials in aHUS. There have been few serious side effects and no reports of tachphylaxis or drug resistance. The results are very encouraging and eculizumab is now recognized as the treatment of choice for aHUS.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.