Prophylactic eculizumab for kidney transplantation in a child with atypical hemolytic uremic syndrome due to complement factor H mutation

Research output: Contribution to journalArticlepeer-review

7 Scopus citations

Abstract

We present a case of successful deceased-donor kidney transplantation in a three-yr-old child with aHUS due to complement factor H mutation, using only prophylactic eculizumab treatment prior to transplant. She developed disease exacerbation in the immediate post-operative period despite having therapeutic eculizumab concentrations and evidence for complete complement pathway blockade. The patient responded well to additional doses of eculizumab and has maintained excellent graft function and disease control in the first year post-transplantation. The optimal dosing scheme for eculizumab in the perioperative period remains to be determined. More sensitive biomarkers of early disease activity are needed to improve disease monitoring. Finally, the duration of eculizumab therapy in patients with aHUS remains to be determined.

Original languageEnglish (US)
Pages (from-to)E185-E189
JournalPediatric Transplantation
Volume18
Issue number6
DOIs
StatePublished - Sep 2014

Keywords

  • atypical hemolytic uremic syndrome
  • complement factor H
  • eculizumab
  • pediatric kidney transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

Fingerprint Dive into the research topics of 'Prophylactic eculizumab for kidney transplantation in a child with atypical hemolytic uremic syndrome due to complement factor H mutation'. Together they form a unique fingerprint.

Cite this