Complement Factor H and Complement Factor H-Related Protein 5 Mutations Associated with Atypical Hemolytic Uremic Syndrome in a Systemic Lupus Erythematosus Patient: Efficacy of Eculizumab


Ersan S., Erkul B., Avcioglu Yilmaz B., GÜLLE S., Yilmaz Z.

TURKISH NEPHROLOGY DIALYSIS AND TRANSPLANTATION JOURNAL, cilt.27, sa.3, ss.308-312, 2018 (ESCI) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 27 Sayı: 3
  • Basım Tarihi: 2018
  • Doi Numarası: 10.5262/tndt.2018.2652
  • Dergi Adı: TURKISH NEPHROLOGY DIALYSIS AND TRANSPLANTATION JOURNAL
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.308-312
  • Anahtar Kelimeler: SLE, aHUS, Eculizumab, THROMBOTIC THROMBOCYTOPENIC PURPURA, ANEMIA, NEPHRITIS, GENES, AHUS
  • Dokuz Eylül Üniversitesi Adresli: Hayır

Özet

Atypical hemolytic uremic syndrome (aHUS) is a disorder characterized by a propensity to thrombotic microangiopathy (TMA) due to defective regulation of the alternative complement pathway. Mutations in genes encoding complement factor H (CFH) and complement factor H-related proteins (CH IR) can be related with aHUS and identified in patients with rheumatologic diseases.