Turkish pediatric atypical hemolytic uremic syndrome registry: initial analysis of 146 patients

BESBAS N., GULHAN B., SOYLEMEZOGLU O., OZCAKAR Z. B., KORKMAZ E., HAYRAN M., ...Daha Fazla

BMC NEPHROLOGY, cilt.18, 2017 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 18
  • Basım Tarihi: 2017
  • Doi Numarası: 10.1186/s12882-016-0420-6
  • Dergi Adı: BMC NEPHROLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Anahtar Kelimeler: Atypical hemolytic uremic syndrome, Turkish registry, Treatment, Outcome, Prognosis, COMPLEMENT INHIBITOR ECULIZUMAB, FACTOR-H MUTATIONS, CLINICAL PHENOTYPE, GENE, VARIANTS, CHILDREN, AHUS, DISCONTINUATION
  • Hacettepe Üniversitesi Adresli: Evet

Özet

Background: Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant morbidity and mortality. Its genetic heterogeneity impacts its clinical presentation, progress, and outcome, and there is no consensus on its clinical management.