Is eculizumab indicated in patients with atypical hemolytic uremic syndrome already on prolonged dialysis? A case report and review of the literature

Pediatr Nephrol. 2019 Dec;34(12):2601-2604. doi: 10.1007/s00467-019-04341-4. Epub 2019 Sep 13.

Abstract

Background: Eculizumab has caused a revolution in the treatment and prognosis of atypical hemolytic uremic syndrome. Early initiation of treatment is recommended to increase chances of renal recovery.

Case-diagnosis/treatment: We describe a boy with atypical hemolytic uremic syndrome who started eculizumab therapy after being on dialysis for 4.5 months, with complete anuria. With treatment, he was weaned off dialysis.

Conclusion: We review the evidence in the literature and discuss the possible mechanism by which eculizumab induces renal recovery even in patients already on prolonged dialysis. This case report highlights the importance of a treatment trial with eculizumab, even in patients already on prolonged dialysis.

Keywords: Child; Dialysis; Eculizumab; aHUS.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antibodies, Monoclonal, Humanized / therapeutic use*
  • Atypical Hemolytic Uremic Syndrome / diagnosis
  • Atypical Hemolytic Uremic Syndrome / drug therapy*
  • Child, Preschool
  • Complement Inactivating Agents / therapeutic use*
  • Female
  • Humans
  • Infant
  • Male
  • Renal Dialysis*
  • Treatment Outcome

Substances

  • Antibodies, Monoclonal, Humanized
  • Complement Inactivating Agents
  • eculizumab