Journal of Investigative Medicine High Impact Case Reports (Nov 2017)

A Case of Hemolytic Uremic Syndrome in a 10-Year-Old Male With Severe Neurologic Involvement Successfully Treated With Eculizumab

  • Malia Rasa MD,
  • James Musgrave MD,
  • Keith Abe MD,
  • Len Tanaka MD,
  • Konstantine Xoinis MD,
  • Bruce Shiramizu MD,
  • Gretchen Foskett MD,
  • Rhiana Lau MD

DOI
https://doi.org/10.1177/2324709617741144
Journal volume & issue
Vol. 5

Abstract

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Hemolytic uremic syndrome (HUS) can be classified as typical and atypical, and the treatment recommendations currently differ between the 2 types. Eculizumab is recommended as first-line treatment for atypical HUS; however, its use in typical HUS has been controversial. We report a case of a 10-year-old male with severe neurologic impairment who was successfully treated with eculizumab, which was started 4 days after onset of neurologic symptoms. Our case supports the use of eculizumab in typical HUS with neurologic involvement, even when given later in the course, as the pathophysiology of typical HUS has been shown to involve activation of the complement pathway, similar to atypical HUS. Further studies are required to establish the efficacy and duration of eculizumab use in this patient population.