ReviewJournal of clinical medicine2025
Atypical Hemolytic Uremic Syndrome: A Review of Complement Dysregulation, Genetic Susceptibility and Multiorgan Involvement.
Review in Journal of clinical medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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.
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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.
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Who cites it
22 citing papers in PubMed.
- Review
- 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
- Complement-mediated thrombotic microangiopathy presenting as atypical hemolytic uremic syndrome during disease-modifying therapy for multiple sclerosis.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026Article
- Comprehensive Prediction Analysis of Novel Noncoding Regulatory Variants Identified in the MicroRNA Binding Regions in Complement System Genes.International journal of molecular sciences · 2026Article
- The complement system in Alzheimer's disease: evaluating biomarker potential in a complex neuroimmune context.Journal of neuroinflammation · 2026Review
- Bridging structure and function: artificial intelligence-based modelling of kidney proteins.Nature reviews. Nephrology · 2026Review
- A review of genetic and epigenetic biomarkers involved in the occurrence of atypical hemolytic uremic syndrome and its therapeutic strategies.Molecular biology reports · 2026Review
- Erythrodermic psoriasis complicated by immune complex-mediated crescentic glomerulonephritis and atypical hemolytic uremic syndrome: a case report.BMC nephrology · 2026Article
- Comprehensive gene profiling by Next-Generation sequencing in a cohort of Egyptian pediatric Atypical HUS.Journal, genetic engineering & biotechnology · 2026Article
- Genetic Insights into Circulating Complement Proteins in Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: A Potential Inflammatory Subgroup.International journal of molecular sciences · 2026Article
- From dog bite to dialysis: complement-mediated haemolytic uraemic syndrome .BMJ case reports · 2026Article
- The Spectrum of Motor Disorders in Patients with Chronic Kidney Disease: Pathogenic Mechanisms, Clinical Manifestations, and Therapeutic Strategies.Journal of clinical medicine · 2026Review
- Complement C5 inhibition in generalized myasthenia gravis is associated with improved survival and increased cardiovascular risk.Frontiers in immunology · 2026Article
- Case Report: Uremic encephalopathy in acute kidney injury caused by abdominal sepsis.Frontiers in radiology · 2026Article
- Eculizumab-Induced Acute Heart Failure Decompensation in a Patient with Clinically Suspected Complement-Mediated Thrombotic Microangiopathy: A Case Report.Infection and drug resistance · 2026Article
- Recurrent Postpartum Atypical Hemolytic Uremic Syndrome Caused by a C3 Pathogenic Variant: A Case Report.International journal of women's health · 2026Article
- Case Report: Avermectin poisoning-associated hemolytic uremic syndrome.Frontiers in immunology · 2026Article
- Complement as a driver of immune-vascular heterogeneity across preeclampsia subtypes: toward a precision medicine framework.Frontiers in immunology · 2026Review
- Long-term outcome and management of complement-mediated thrombotic microangiopathy/aHUS.Hematology. American Society of Hematology. Education Program · 2025Review
- Demographics and baseline disease characteristics of UK patients within the global aHUS registry.BMC nephrology · 2025Article
Corrections and comments
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Authors and funding
8 authors.
Funding
Abstract
Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA) characterized by complement dysregulation, leading to microvascular thrombosis and multi-organ injury. TMAs are defined by thrombocytopenia, microangiopathic hemolytic anemia and organ dysfunction caused by small-vessel thrombosis. Unlike thrombotic thrombocytopenic purpura, which results from severe ADAMTS13 deficiency, aHUS is driven by uncontrolled activation of the alternative complement pathway. While the kidneys are most frequently affected, other vital organs can also be involved. Genetic susceptibility contributes significantly to disease risk, but a trigger such as infection, pregnancy or autoimmune disease is usually required. Diagnosis is challenging due to overlapping features with other TMAs and relies on exclusion and complement testing. C5 inhibitors, such as eculizumab and ravulizumab, have revolutionized treatment but necessitate prophylactic vaccination and ongoing clinical surveillance. While these therapies provide effective disease control, discontinuing treatment remains complex, especially in patients with complement gene mutations. New therapies targeting various points in the complement cascade are under investigation and may offer safer, more cost-effective options. Progress in genetic profiling and biomarker discovery is essential for earlier diagnosis, individualized therapy and relapse prevention. This review highlights recent advances in the understanding of aHUS pathophysiology, clinical features and evolving therapeutic strategies aimed at improving patient outcomes.
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