Trial reportBlood2014
Dynamics of complement activation in aHUS and how to monitor eculizumab therapy.
Trial report in Blood, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05985122 (Towards the Most Accurate Diagnosis and Monitoring of Complement-mediated Rare Kidney Diseases), which is not on this map. Cited by 170 papers, 2 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.
Towards the Most Accurate Diagnosis and Monitoring of Complement-mediated Rare Kidney Diseases
Who cites it
170 citing papers in PubMed, 2 syntheses or guidelines pooled it, 330 citations in OpenAlex.
- Systematic review of atypical hemolytic uremic syndrome biomarkers.Pediatric nephrology (Berlin, Germany) · 2022Pooled it
- Interventions for atypical haemolytic uraemic syndrome.The Cochrane database of systematic reviews · 2021Pooled it
- Exploratory Prognostic Biomarkers of Complement-Mediated Thrombotic Microangiopathy (CM-TMA) in Adults with Atypical Hemolytic Uremic Syndrome (aHUS): Analysis of a Phase III Study of Ravulizumab.Molecular diagnosis & therapy · 2023Trial
- Trial
- Trial
- IPNA clinical practice recommendations for the management of aHUS and secondary TMA in children.Pediatric nephrology (Berlin, Germany) · 2026Article
- Complement inhibitor therapy in atypical hemolytic uremic syndrome (aHUS): evaluating the economic impact of introducing eculizumab biosimilars in Germany.BMC nephrology · 2026Article
- Article
- Response to the Letter to the Editor Entitled "Defining "Dominance" in Atypical Hemolytic Uremic Syndrome: Distinguishing Amplification From Initiation".Kidney international reports · 2026Article
- Measurement of complement proteins may aid interpretation of pathophysiology in kidney transplant recipients with atypical hemolytic uremic syndrome.CEN case reports · 2026Article
- Comprehensive analysis of complement activation in a hydroxyurea-treated patient cohort with sickle cell disease.Blood advances · 2026Article
- Establishment of a Time-Resolved Fluoroimmunoassay for sC5b-9 and Validation in Lupus Nephritis.Journal of fluorescence · 2026Article
- Therapeutic advances in eculizumab for atypical hemolytic uremic syndrome: a narrative review.Frontiers in pharmacology · 2026Review
- Molecular determinants of STEC-HUS: from complement activation to microvascular thrombosis.Frontiers in immunology · 2026Article
- Comparative Analysis of Clinicopathological Features and Outcomes Between Kidney-Limited and Systemic Thrombotic Microangiopathy: A Study Based on a Semiquantitative Pathological Scoring System.International journal of nephrology and renovascular disease · 2026Article
- Complement diagnostics and therapeutics for the pediatric population: early successes and opportunities for further advancement.Frontiers in pharmacology · 2026Review
- Update in the diagnosis of complement-mediated thrombotic microangiopathy/atypical hemolytic uremic syndrome.Hematology. American Society of Hematology. Education Program · 2025Review
- Long-term outcome and management of complement-mediated thrombotic microangiopathy/aHUS.Hematology. American Society of Hematology. Education Program · 2025Review
- Article
- Soluble C5b-9 (sC5b-9) in Pediatrics-A Clinical Assessment.Journal of clinical laboratory analysis · 2025Article
110 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
16 authors at 6 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Atypical hemolytic-uremic syndrome (aHUS) is associated with genetic complement abnormalities/anti-complement factor H antibodies, which paved the way to treatment with eculizumab. We studied 44 aHUS patients and their relatives to (1) test new assays of complement activation, (2) verify whether such abnormality occurs also in unaffected mutation carriers, and (3) search for a tool for eculizumab titration. An abnormal circulating complement profile (low C3, high C5a, or SC5b-9) was found in 47% to 64% of patients, irrespective of disease phase. Acute aHUS serum, but not serum from remission, caused wider C3 and C5b-9 deposits than control serum on unstimulated human microvascular endothelial cells (HMEC-1). In adenosine 5'-diphosphate-activated HMEC-1, also sera from 84% and 100% of patients in remission, and from all unaffected mutation carriers, induced excessive C3 and C5b-9 deposits. At variance, in most patients with C3 glomerulopathies/immune complex-associated membranoproliferative glomerulonephritis, serum-induced endothelial C5b-9 deposits were normal. In 8 eculizumab-treated aHUS patients, C3/SC5b-9 circulating levels did not change posteculizumab, whereas serum-induced endothelial C5b-9 deposits normalized after treatment, paralleled or even preceded remission, and guided drug dosing and timing. These results point to efficient complement inhibition on endothelium for aHUS treatment. C5b-9 endothelial deposits might help monitor eculizumab effectiveness, avoid drug overexposure, and save money considering the extremely high cost of the drug.
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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.