ArticlePediatric nephrology (Berlin, Germany)2026
STEC-triggered DEAP-HUS in a toddler: successful management with eculizumab and mycophenolate mofetil without corticosteroids.
Article in Pediatric nephrology (Berlin, Germany), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Deficiency of CFHR proteins and autoantibody-positive HUS (DEAP-HUS) is a rare autoimmune subtype of atypical HUS that is distinct from typical Shiga toxin-producing Escherichia coli (STEC)-associated HUS. We report the case of a 30-month-old child initially presenting with STEC-associated HUS whose severe clinical course prompted complement investigation despite confirmed STEC positivity. Markedly elevated anti-factor H antibody titers and a homozygous CFHR1-CFHR3 deletion led to reclassification as DEAP-HUS. The patient was treated with eculizumab without high-dose corticosteroids, followed by mycophenolate mofetil (MMF) introduced at month 5, allowing successful discontinuation of complement blockade at month 9. Anti-factor H antibody titers remained stable throughout follow-up without clinical relapse, suggesting that MMF monotherapy provides sufficient immunological stabilization even in the absence of significant antibody reduction.
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