ReviewJournal of atherosclerosis and thrombosis2019
Pathogenesis of Atypical Hemolytic Uremic Syndrome.
Review in Journal of atherosclerosis and thrombosis, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 62 papers, 1 of them a synthesis that pooled it.
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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
62 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Systematic review of atypical hemolytic uremic syndrome biomarkers.Pediatric nephrology (Berlin, Germany) · 2022Pooled it
- Early Donor-Derived Cell-Free DNA Kinetics After Kidney Transplantation in Atypical Hemolytic Uremic Syndrome: A Preliminary Observational Study.International journal of molecular sciences · 2026Observational
- Atypical Hemolytic Uremic Syndrome Associated With Malignant Hypertension Presenting With Pulmonary-Renal Syndrome-like Symptoms.Kidney medicine · 2026Article
- Impaired cellular trafficking of a thrombomodulin mutant causes severe bleeding, thrombosis, and atypical hemolytic uremic syndrome.Blood vessels, thrombosis & hemostasis · 2026Article
- Beyond complement: comprehensive assessment of factor XIII plasma dynamics and genetics in atypical hemolytic uremic syndrome.Research and practice in thrombosis and haemostasis · 2026Article
- Atypical Hemolytic Uremic Syndrome With Hypocellular Bone Marrow: A Report of a Rare Case.Cureus · 2026Article
- Measurement of complement proteins may aid interpretation of pathophysiology in kidney transplant recipients with atypical hemolytic uremic syndrome.CEN case reports · 2026Article
- Multifactorial chronic kidney disease and the kidney capacity-workload balance.Nature reviews. Nephrology · 2026Review
- Complement-Mediated Kidney Diseases: Role of Alternative Pathway in Glomerular Inflammation.Kidney international reports · 2026Review
- Characteristics and clinical courses of patients with atypical haemolytic uraemic syndrome on dialysis withdrawal after eculizumab treatment: sub-analysis of post-marketing surveillance in Japan.Journal of nephrology · 2025Article
- Iptacopan in C5 blockade refractory atypical hemolytic uremic syndrome with associated Castleman's disease: case report.BMC nephrology · 2025Article
- Management of Atypical Haemolytic Uraemic Syndrome With Triggers: Diagnostic and Treatment Algorithms From an Asia-Pacific Perspective.Nephrology (Carlton, Vic.) · 2025Review
- Atypical hemolytic uremic syndrome treated with anti-C5 antibody agent eculizumab, without genetic complement abnormalities.Nagoya journal of medical science · 2025Article
- The Hideous Side of Acute Pancreatitis: A Case of Pancreatitis-Induced Atypical Hemolytic Uremic Syndrome.Cureus · 2025Article
- Potentially reversible severe cardiac involvement in thrombotic microangiopathies with malignant hypertension.Journal of nephrology · 2025Article
- Article
- Atypical Hemolytic Uremic Syndrome: A Review of Complement Dysregulation, Genetic Susceptibility and Multiorgan Involvement.Journal of clinical medicine · 2025Review
- New-onset hematologic disorders following COVID-19 vaccination: a systematic review.Clinical and experimental vaccine research · 2025Article
- The Path to Accessible Care: Development and Impact of Eculizumab Biosimilars for Paroxysmal Nocturnal Hemoglobinuria and Atypical Hemolytic Uremic Syndrome.BioDrugs : clinical immunotherapeutics, biopharmaceuticals and gene therapy · 2025Review
- A Case of Atypical Hemolytic Uremic Syndrome With a Complement Factor I Mutation Triggered by a Femoral Neck Fracture.Nephrology (Carlton, Vic.) · 2025Article
2 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Atypical hemolytic uremic syndrome (aHUS) is a type of thrombotic microangiopathy (TMA) defined by thrombocytopenia, microangiopathic hemolytic anemia, and renal failure. aHUS is caused by uncontrolled complement activation in the alternative pathway (AP). A variety of genetic defects in complement-related factors or acquired autoantibodies to the complement regulators have been found in 50 to 60% of all cases. Recently, however, the classification and diagnosis of aHUS are becoming more complicated. One reason for this is that some factors, which have not been regarded as complement-related factors, have been reported as predisposing factors for phenotypic aHUS. Given that genotype is highly correlated with the phenotype of aHUS, careful analysis of underlying genetic abnormalities or acquired factors is needed to predict the prognosis or to decide an optimal treatment for the disease. Another reason is that complement dysregulation in the AP have also been found in a part of other types of TMA such as transplantation-related TMA and pregnancy-related complication. Based on these findings, it is now time to redefine aHUS according to the genetic or acquired background of abnormalities.Here, we review the pathogeneses and the corresponding phenotypes of aHUS and complement-related TMAs.
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