Neurological Involvement in Children with Hemolytic Uremic Syndrome

Abstract

Our objective was to establish the rate of neurological involvement in STEC-HUS and describe the clinical presentation, management and outcome. A retrospective chart review of children aged ≤16 years with STEC-HUS in Children’s Health Ireland from 2005 to 2018 was conducted. Laboratory confirmation of STEC infection was required for inclusion. Neurological involvement was defined as encephalopathy, focal neurological deficit and/or seizure activity. Data on clinical presentation, management and outcome were collected. We identified 240 children with HUS; 202 had confirmed STEC infection. Neurological involvement occurred in 22 (10.9%). The most common presentation was seizures (72.7%). In the neurological group, 19 (86.4%) were treated with plasma exchange and/or Eculizumab. Of the 21 surviving children with neurological involvement, 19 (90.5%) achieved a complete neurological recovery. A higher proportion of children in the neurological group had renal sequelae (26.6% vs. 11.5 %, P=.031). One patient died from multi-organ failure. Conclusion: We have identified the rate of neurological involvement in a large cohort of children with STEC-HUS as 10.9%. Neurological involvement in STEC-HUS is associated with good long-term outcome (complete neurological recovery in 90.5%) and a low case-fatality rate (4.5%) in our cohort.