Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi. (Turkish)

Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment. (English)

Atypical hemolytic uremic syndrome (aHUS) is a rare, chronic disease with frequent progression to end-stage renal disease. In the majority of these patients, there is a genetic or acquired disorder that causes the dysregulation of the alternative complement pathway. Factor H autobodies have been reported in 6-11% of the patients with aHUS. We aimed to present a case with anti-FH antibodyassociated aHUS with intractable relapses despite plasma exchange and immunosuppressive treatment, but remission was provided by the use of eculizumab. [ABSTRACT FROM AUTHOR]

Atipik hemolitik üremik sendrom (aHÜS), sıklıkla son dönem böbrek yetersizliğine ilerleyen ender görülen kronik bir hastalıktır. Bu hastaların çoğunda alternatif kompleman yolağının bozulmasına neden olan genetik veya edinsel bozukluk vardır. Kompleman Faktör H otoantikorları aHÜS'lü vakaların %6-11'inde bildirilmiştir. Plazma değişimi ve immunsupresif tedaviye rağmen, relapsları önlenemeyen, ancak Ekulizumab kullanımı ile remisyon sağlanan bir anti-FH antikor ilişkili aHÜS vakasını sunmayı amaçladık. [ABSTRACT FROM AUTHOR]

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