2017Çocuk Dergisi / Journal of ChildOpen access

Complement factor H antibody-associated atypical hemolytic uremic syndrome and eculizumab treatment

Zeynep Yürük Yıldırım, Alev Yılmaz, Sevinç Emre, İlmay Bilge, Bağdagül Aksu, Aydan Şirin

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Abstract

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.

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What this paper is about

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.

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Available abstract

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.

Key concepts: Atypical hemolytic uremic syndrome, Eculizumab, Medicine, Complement system, Thrombotic microangiopathy, Factor H, Immunology, Alternative complement pathway

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