ArticlePediatric nephrology (Berlin, Germany)2026
Incidence and severity of extrarenal manifestations and outcomes in pediatric hemolytic uremic syndrome: a retrospective cohort study.
Article in Pediatric nephrology (Berlin, Germany), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Constructing unbiased pediatric cohorts in TriNetX.Pediatric nephrology (Berlin, Germany) · 2026Article
- Balancing generalizability and internal validity in pediatric TriNetX cohort design.Pediatric nephrology (Berlin, Germany) · 2026Article
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10 authors.
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Abstract
backgroundPediatric hemolytic uremic syndrome (HUS) is characterized by acute kidney injury (AKI), thrombocytopenia, and microangiopathic hemolytic anemia. While kidney involvement is well recognized, extrarenal manifestations of HUS remain poorly characterized.
methodsWe conducted a retrospective cohort study using TriNetX, a global electronic health record database, to examine the incidence of extrarenal complications and clinical outcomes in children (< 18 years) with HUS. Patients were identified via ICD-10-CM codes from 2000 to 2024. Primary outcomes included the 1-year cumulative incidence of extrarenal manifestations, mortality, and kidney disease progression.
resultsA total of 2362 pediatric patients with HUS were included (mean age 4.04 ± 3.65 years; 47.8% male). At 1 year, hypertensive disease was the most prevalent extrarenal manifestation (24.9%), followed by respiratory, gastrointestinal, neurologic, and cardiovascular complications. Patients with "other" HUS (ICD-10 D59.39) experienced higher rates of extrarenal disease compared to infection-associated or hereditary HUS. Mortality was 2.2% at 1 year, with most deaths occurring within 3 months of diagnosis. AKI occurred in 34.0%, chronic kidney disease (CKD) in 14.6%, and kidney failure in 4.0% of patients. One-year hospitalization, ICU admission, and mechanical ventilation rates were 34.8%, 6.7%, and 5.8%, respectively. After the publication of the 2015 treatment guidelines for HUS, eculizumab use rose significantly; 12.7% of patients received plasmapheresis.
conclusionsIn our cohort, pediatric HUS was associated with significant short- and medium-term extrarenal morbidity. These findings highlight the need for vigilant long-term follow-up and refined treatment strategies targeting extrarenal disease burden in pediatric HUS.
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