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Background <p>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.</p> Methods <p>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 (&lt; 18&#xa0;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.</p> Results <p>A total of 2362 pediatric patients with HUS were included (mean age 4.04 ± 3.65&#xa0;years; 47.8% male). At 1&#xa0;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&#xa0;year, with most deaths occurring within 3&#xa0;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.</p> Conclusions <p>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.</p> Graphical Abstract <p></p>

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

  • 期刊论文

  • 发表日期：

    2025年12月9日

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

摘要

Background

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.

Methods

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.

Results

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.

Conclusions

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.

Graphical Abstract