Observational studyJournal of nephrology2025
Clinical and genetic characteristics of patients diagnosed with atypical hemolytic uremic syndrome (aHUS): epidemiological data from the Belgian cohort of the Global aHUS Registry.
Observational study in Journal of nephrology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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Who cites it
3 citing papers in PubMed.
- Analysis of causes and outcomes of principal secondary thrombotic microangiopathy: a 9-year cohort study from a tertiary pediatric center in China.Research and practice in thrombosis and haemostasis · 2026Article
- A review of genetic and epigenetic biomarkers involved in the occurrence of atypical hemolytic uremic syndrome and its therapeutic strategies.Molecular biology reports · 2026Review
- Complement Dysregulation in Kidney Diseases: Mechanisms, Biomarkers, and Emerging Targeted Therapies.International journal of molecular sciences · 2026Review
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Authors and funding
17 authors.
Funding
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Abstract
backgroundAtypical hemolytic uremic syndrome (aHUS) usually results from an overactivation of the alternative complement pathway. As large clinical trials are scarce, patient registries can partially fill the knowledge gap on patient characteristics, management, and outcomes. We here describe the baseline clinical and genetic characteristics as well as the management of all Belgian patients enrolled in the Global aHUS Registry at data cut-off.
methodsThis observational study prospectively and retrospectively collected data (data cut-off: December 26, 2022) from patients of all ages with a clinical diagnosis of aHUS, irrespective of treatment.
resultsA total of 121 Belgian patients were registered in the Global aHUS Registry, resulting in a prevalence of 10.4 aHUS patients per million inhabitants, with a higher proportion of females affected (57.9% vs 42.1% of males). Among the 109 patients tested for at least one variant and/or anti-complement factor H (CFH) antibodies, 36 were positive for a pathogenic complement gene variant associated with aHUS (n = 29) and/or seropositive for anti-CFH antibodies (n = 14). The most common variants affected CFH, C3 and CD46. The higher proportion of complement gene variants in treated women versus men was not related to a specific gene.
conclusionsThis study strengthens the real-world evidence on aHUS and adds to previously published Global aHUS Registry data. In addition, it provides insights into the differential epidemiology of the disease in Belgium and demonstrates the increased susceptibility of women to aHUS across the whole spectrum of recognized complement gene variants.
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