Extra-Renal manifestations of atypical hemolytic uremic syndrome in children


Fidan K., Goknar N., GÜLHAN B., MELEK E., Yildirim Z. Y. , Baskin E., ...More

PEDIATRIC NEPHROLOGY, vol.33, no.8, pp.1395-1403, 2018 (Peer-Reviewed Journal) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 33 Issue: 8
  • Publication Date: 2018
  • Doi Number: 10.1007/s00467-018-3933-3
  • Journal Name: PEDIATRIC NEPHROLOGY
  • Journal Indexes: Science Citation Index Expanded, Scopus
  • Page Numbers: pp.1395-1403
  • Keywords: Atypical hemolytic uremic syndrome, Children, Neurological involvement, Extrarenal involvement, Eculizumab, ECULIZUMAB, COMPLEMENT, MUTATIONS, INVOLVEMENT

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood.