Neurological involvement in children with hemolytic uremic syndrome

Autor: Eilish Twomey, Michael Riordan, CS Costigan, Atif Awan, Tara Raftery, Bryan Lynch, Dermot Michael Wildes, Anne G Carroll, Kathleen M. Gorman, M. Waldron, Robert Cunney, Clodagh Sweeney, Amre Shahwan, Niamh Marie Dolan, Claire Reynolds, Maria Stack, Declan O'Rourke, Richard J. Drew
Rok vydání: 2021
Předmět:
Zdroj: European Journal of Pediatrics. 181:501-512
ISSN: 1432-1076
0340-6199
DOI: 10.1007/s00431-021-04200-1
Popis: Our objective was to establish the rate of neurological involvement in Shiga toxin-producing Escherichia coli–hemolytic uremic syndrome (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 (11%). The most common presentation was seizures (73%). In the neurological group, 19 (86%) were treated with plasma exchange and/or eculizumab. Of the 21 surviving children with neurological involvement, 19 (91%) achieved a complete neurological recovery. A higher proportion of children in the neurological group had renal sequelae (27% vs. 12%, 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 11%. Neurological involvement in STEC-HUS is associated with good long-term outcome (complete neurological recovery in 91%) and a low case-fatality rate (4.5%) in our cohort. What is Known:• HUS is associated with neurological involvement in up to 30% of cases.• Neurological involvement has been reported as predictor of poor outcome, with associated increased morbidity and mortality. What is New:• The incidence of neurological involvement in STEC-HUS is 11%.• Neurological involvement is associated with predominantly good long-term outcome (90%) and a reduced case-fatality rate (4.5%) compared to older reports.
Databáze: OpenAIRE
Externí odkaz: