Could plasma based therapies still be considered in selected cases with atypical hemolytic uremic syndrome?

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ÖZLÜ S. G., GÜLHAN B., Aydog O., Atayar E., Delibas A., Parmaksiz G., ...Daha Fazla

TURKISH JOURNAL OF PEDIATRICS, cilt.63, sa.6, ss.986-993, 2021 (SCI-Expanded) identifier identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 63 Sayı: 6
  • Basım Tarihi: 2021
  • Doi Numarası: 10.24953/turkjped.2021.06.006
  • Dergi Adı: TURKISH JOURNAL OF PEDIATRICS
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.986-993
  • Anahtar Kelimeler: atypical hemolytic uremic syndrome, treatment, plasma infusion, plasma exchange, outcome, CLINICAL CHARACTERISTICS, COMPLEMENT, MUTATIONS, ECULIZUMAB, MANAGEMENT, IMPACT, MICROANGIOPATHY, AHUS
  • Hacettepe Üniversitesi Adresli: Evet

Özet

Background. Atypical hemolytic uremic syndrome (aHUS) occurs due to defective regulation of the alternative complement pathway (ACP) on vascular endothelial cells. Plasma based therapy (PT) was the mainstay of the treatment for aHUS for many years until the introduction of therapies targeting blockage of the complement system. The aim of this study was to evaluate patients with aHUS who had been treated with plasma based therapies alone.