1. In a large multi-center study of children with hemolytic uremic syndrome, those with any acute neurologic manifestation (ANM) had increased risk of mortality.
2. Brain infarction, brain hemorrhage, anoxic brain injury, and brain edema were independently associated with mortality.
Evidence Rating Level: 2 (Good)
Study Rundown: Hemolytic uremic syndrome (HUS) is a microangiopathic hemolytic anemia characterized by anemia, thrombocytopenia, and renal dysfunction. One of the most serious complications of HUS is neurologic injury, which can lead to devastating sequelae including death. Previously, only small studies examined acute neurologic manifestations (ANMs) of HUS, with widely varying conclusions. This study characterized ANMs and their association with in-hospital mortality in nearly 4,000 children with HUS using a database containing information from over 40 children’s hospitals in North America. Overall, ANMs occurred in 10.4% of patients. Mortality was significantly higher in patients with any ANM (13.9%) compared to those without an ANM (1.8%). Furthermore, mortality was higher in patients with ≥ 2 ANMs (17.6%) than in those with 1 ANM (11.9%). Researchers also examined risk of mortality with specific ANMs and specific combinations of ANMs. One important limitation of this study was the lack of differentiation between typical and atypical HUS, which are considered to be different disease processes. Overall, this robust analysis of data obtained from a large database provides valuable information in identifying which children with HUS are at risk for worse outcomes.
In-Depth [retrospective cohort]: Data was obtained from the Pediatric Health Information System database, which contains information from over 40 tertiary children’s hospitals in North America. Overall, 3915 children (52.5% female, 75.5% white, median age 3.8 years) with HUS were included in the study. The median age of patients with ANMs was 3.3 years. In addition to increased mortality in patients with ANMs, average length of stay was also longer in those with ANMs compared to those without (27.8 vs. 13.8 days, p<0.001). The three most common ANMs were encephalopathy (60% of all patients with ANMs), seizures (26.4%), and stroke (22.5%). Mortality varied between specific ANMs – encephalopathy (4.3%), seizures (8.9%), meningitis (21.7%), stroke (22.2%), intracranial hemorrhage (40%), cerebral edema (25%), and anoxic brain injury (40%). Patients < 30 days old were at increased risk of mortality, as were those who required mechanical ventilation or ECMO. ANMs independently associated with mortality were brain infarction (OR 2.64, p=0.03), brain hemorrhage (OR 3.09, p=0.005), anoxic brain injury (OR 3.92, p=0.006), and brain edema (OR 4.81, p=0.002).
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