ArticleJournal of nephrology2023
Clinical characteristics and outcomes of a patient population with atypical hemolytic uremic syndrome and malignant hypertension: analysis from the Global aHUS registry.
Article in Journal of nephrology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01522183 (An Observational, Non-Interventional, Multi-Center, Multi-National Study of Patients With Atypical Hemolytic-Uremic Syndrome), which is not on this map. Cited by 18 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.
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.
An Observational, Non-Interventional, Multi-Center, Multi-National Study of Patients With Atypical Hemolytic-Uremic Syndrome (aHUS Registry)
Who cites it
18 citing papers in PubMed, 25 citations in OpenAlex.
- Long-term renal outcomes of patients with biopsy-proven malignant hypertension-associated renal thrombotic microangiopathy who are dialysis-independent at baseline.Renal failure · 2026Observational
- Complement Dysregulation in Kidney Diseases: Mechanisms, Biomarkers, and Emerging Targeted Therapies.International journal of molecular sciences · 2026Review
- Long-term outcome and management of complement-mediated thrombotic microangiopathy/aHUS.Hematology. American Society of Hematology. Education Program · 2025Review
- Significant proteinuria as a predictor of renal prognosis in malignant hypertension patients with thrombotic microangiopathy: a prospective cohort study.BMC nephrology · 2025Observational
- Management of Atypical Haemolytic Uraemic Syndrome With Triggers: Diagnostic and Treatment Algorithms From an Asia-Pacific Perspective.Nephrology (Carlton, Vic.) · 2025Review
- Demographics and baseline disease characteristics of UK patients within the global aHUS registry.BMC nephrology · 2025Article
- Potentially reversible severe cardiac involvement in thrombotic microangiopathies with malignant hypertension.Journal of nephrology · 2025Article
- Haemolytic uremic syndrome as a cause of chronic kidney disease stage 5 in children is in retreat: results from the Polish Registry of Kidney Replacement Therapy in children (2000-2023).Pediatric nephrology (Berlin, Germany) · 2025Article
- Kidney outcomes of malignant hypertension-associated thrombotic microangiopathy in patients with and without IgA nephropathy: a propensity score-matched analysis.Clinical kidney journal · 2025Article
- Complement-Mediated Kidney Disease and Living Donor Transplantation: Tailoring Approaches to Improve Outcomes.Current transplantation reports · 2025Article
- Real-World effectiveness of eculizumab in atypical hemolytic uremic syndrome: a retrospective study from Western China.Frontiers in endocrinology · 2025Article
- Glucose-6-Phosphate Dehydrogenase Deficiency Presenting as Atypical Hemolytic Uremic Syndrome: A Case Series and Literature Review.Case reports in nephrology · 2025Article
- Glomerular Hematuria as a Predictor of Renal Prognosis in Malignant Hypertension Patients with Thrombotic Microangiopathy: A Propensity Score-Matched Analysis of a Biopsy-Based Cohort Study.Kidney diseases (Basel, Switzerland) · 2024Article
- Eculizumab for adult patients with atypical haemolytic-uraemic syndrome: full dataset analysis of Japanese post-marketing surveillance.Journal of nephrology · 2024Article
- Article
- Vascular injury in glomerulopathies: the role of the endothelium.Frontiers in nephrology · 2024Review
- Thrombotic microangiopathies after kidney transplantation in modern era: nosology based on chronology.BMC nephrology · 2023Article
- Propensity score-matched analysis of long-term outcomes for living kidney donation in alternative complement pathway diseases: a pilot study.Journal of nephrology · 2023Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
10 authors at 10 institutions in 7 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionAtypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) often caused by alternative complement dysregulation. Patients with aHUS can present with malignant hypertension (MHT), which may also cause TMA.
methodsThis analysis of the Global aHUS Registry (NCT01522183) assessed demographics and clinical characteristics in eculizumab-treated and not-treated patients with aHUS, with (n = 71) and without (n = 1026) malignant hypertension, to further elucidate the potential relationship between aHUS and malignant hypertension.
resultsWhile demographics were similar, patients with aHUS + malignant hypertension had an increased need for renal replacement therapy, including kidney transplantation (47% vs 32%), and more pathogenic variants/anti-complement factor H antibodies (56% vs 37%) than those without malignant hypertension. Not-treated patients with malignant hypertension had the highest incidence of variants/antibodies (65%) and a greater need for kidney transplantation than treated patients with malignant hypertension (65% vs none). In a multivariate analysis, the risk of end-stage kidney disease or death was similar between not-treated patients irrespective of malignant hypertension and was significantly reduced in treated vs not-treated patients with aHUS + malignant hypertension (adjusted HR (95% CI), 0.11 [0.01-0.87], P = 0.036).
conclusionsThese results confirm the high severity and poor prognosis of untreated aHUS and suggest that eculizumab is effective in patients with aHUS ± malignant hypertension. Furthermore, these data highlight the importance of accurate, timely diagnosis and treatment in these populations and support consideration of aHUS in patients with malignant hypertension and TMA. TRIAL REGISTRATION DETAILS: Atypical Hemolytic-Uremic Syndrome (aHUS) Registry. Registry number: NCT01522183 (first listed 31st January, 2012; start date 30th April, 2012).
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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.