Incidence and severity of extrarenal manifestations and outcomes in pediatric hemolytic uremic syndrome: a retrospective cohort study

Rupesh Raina; Keval Yerigeri; Kush Doshi; Amanda Osagie-Ogbeide; Imad Haq; Sydney Smith; Priyanka Khandelwal; Christoph Licht; Carla Nester; Olivia Boyer

doi:10.1007/s00467-025-07079-4

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Incidence and severity of extrarenal manifestations and outcomes in pediatric hemolytic uremic syndrome: a retrospective cohort study

Journal article

Peer reviewed

Incidence and severity of extrarenal manifestations and outcomes in pediatric hemolytic uremic syndrome: a retrospective cohort study

Rupesh Raina, Keval Yerigeri, Kush Doshi, Amanda Osagie-Ogbeide, Imad Haq, Sydney Smith, Priyanka Khandelwal, Christoph Licht, Carla Nester and Olivia Boyer

Pediatric nephrology (Berlin, West), Vol.41(6), pp.1693-1704

06/2026

DOI: 10.1007/s00467-025-07079-4

PMID: 41364181

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Abstract

Pediatric 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. We 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. A 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. In 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.

Hemolytic uremic syndrome

Acute kidney injury

Eculizumab

Chronic kidney disease

Complement system

Shiga toxins

Child

Details

Title: Subtitle: Incidence and severity of extrarenal manifestations and outcomes in pediatric hemolytic uremic syndrome: a retrospective cohort study
Creators: Rupesh Raina - Cleveland Clinic Akron General
Keval Yerigeri - Case Western Reserve University
Kush Doshi - Cleveland Clinic Akron General
Amanda Osagie-Ogbeide - Northeast Ohio Medical University
Imad Haq - Wright State University
Sydney Smith - Northeast Ohio Medical University
Priyanka Khandelwal - All India Institute of Medical Sciences
Christoph Licht - Hospital for Sick Children
Carla Nester - University of Iowa
Olivia Boyer - Institut des Maladies Génétiques Imagine
Resource Type: Journal article
Publication Details: Pediatric nephrology (Berlin, West), Vol.41(6), pp.1693-1704
DOI: 10.1007/s00467-025-07079-4
PMID: 41364181
NLM abbreviation: Pediatr Nephrol
ISSN: 1432-198X
eISSN: 1432-198X
Publisher: Springer Nature
Language: English
Electronic publication date: 12/09/2025
Date published: 06/2026
Academic Unit: Nephrology, Dialysis and Transplantation; Stead Family Department of Pediatrics; Internal Medicine
Record Identifier: 9985090591602771

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