Neonatal onset atypical hemolytic uremic syndrome successfully treated with eculizumab

Besbas N., Gulhan B., Karpman D., Topaloglu R., Duzova A., Korkmaz E., ...More

PEDIATRIC NEPHROLOGY, vol.28, no.1, pp.155-158, 2013 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 28 Issue: 1
  • Publication Date: 2013
  • Doi Number: 10.1007/s00467-012-2296-4
  • Journal Name: PEDIATRIC NEPHROLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.155-158
  • Keywords: Atypical hemolytic uremic syndrome, Complement factor H mutation, Eculizumab, Newborn, MUTATIONS
  • Hacettepe University Affiliated: Yes

Abstract

Atypical hemolytic uremic syndrome (aHUS) is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Neonatal cases are extremely uncommon. Plasma therapy is the first choice therapy in patients with aHUS based on the belief of an underlying complement dysregulation. Alternatively, eculizumab, which targets complement 5, is used to block complement activation.