These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Eculizumab in STEC-HUS: a paradigm shift in the management of pediatric patients with neurological involvement.
    Author: Wildes DM, Harvey S, Costigan CS, Sweeney C, Twomey É, Awan A, Gorman KM.
    Journal: Pediatr Nephrol; 2024 Jan; 39(1):315-324. PubMed ID: 37491519.
    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.
    [Abstract] [Full Text] [Related] [New Search]