We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Eculizumab in STEC-HUS: a paradigm shift in the management of pediatric patients with neurological involvement.
- Authors
Wildes, Dermot Michael; Harvey, Susan; Costigan, Caoimhe Suzanne; Sweeney, Clodagh; Twomey, Éilis; Awan, Atif; Gorman, Kathleen Mary
- Abstract
Background: Eculizumab for the treatment of atypical hemolytic uremic syndrome (HUS) is a standard of care. Central nervous system (CNS) involvement in Shiga toxin–producing Escherichia coli (STEC)-HUS is associated with increased morbidity and mortality. There is no consensus on the use of plasma exchange and/or eculizumab. We report a series (n = 4) of children with CNS involvement in STEC-HUS with excellent outcomes after treatment with eculizumab only and supportive therapies. Methods: A retrospective chart review of patients with CNS involvement in STEC-HUS is managed with supportive therapies and eculizumab only. Results: Four patients (75% female) with a median age of 5 years and 11 months (IQR: 23.5–105.5 months) were admitted to a tertiary pediatric nephrology center with CNS involvement in STEC-HUS. Neurological symptoms presented between days 2 and 7 of illness and included ataxia, altered mental status, visual symptoms, and seizures. All had an abnormal MRI brain. All received two doses of eculizumab, 1 week apart (dosing according to weight). Resolution of neurological symptoms was evident at a mean of 60 h post-administration (range: 24–72 h). All patients have complete kidney and neurological recovery at 12-month follow-up. Conclusion: We present a case series of four children with STEC-HUS and CNS involvement, managed with eculizumab only, in lieu of plasma exchange (as per our previous policy). The marked improvement in symptoms in our cohort supports the use of eculizumab, rather than plasma exchange in the CNS involvement of STEC-HUS.
- Subjects
THERAPEUTIC use of monoclonal antibodies; ESCHERICHIA coli; CONVALESCENCE; PLASMA exchange (Therapeutics); PEDIATRICS; RETROSPECTIVE studies; ACQUISITION of data; TERTIARY care; MAGNETIC resonance imaging; MONOCLONAL antibodies; PARADIGMS (Social sciences); NEPHROLOGY; COMPARATIVE studies; MEDICAL records; DESCRIPTIVE statistics; CASE studies; HEMOLYTIC-uremic syndrome; BACTERIAL toxins; SEIZURES (Medicine); CENTRAL nervous system; ATAXIA
- Publication
Pediatric Nephrology, 2024, Vol 39, Issue 1, p315
- ISSN
0931-041X
- Publication type
Article
- DOI
10.1007/s00467-023-06102-w