Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment

Yürük Yıldırım Zeynep; Yılmaz Alev; Emre Sevinç; Bilge Ilmay; Yavaş Aksu Bağdagül; Şirin Aydan

doi:https://dx.doi.org/10.5222/j.child.2017.089

Case Report

DOI :10.5222/j.child.2017.089 IUP :10.5222/j.child.2017.089 Full Text (PDF)

Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment

Zeynep Nagehan Yürük Yıldırım, Alev Yılmaz, Sevinç Emre, Ilmay Bilge, Bağdagül Yavaş Aksu, Aydan Şirin

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.

Keywords: Atypical hemolytic uremic syndrome, factor H autoantibodies, eculizumab therapy

DOI :10.5222/j.child.2017.089 IUP :10.5222/j.child.2017.089 Full Text (PDF)

Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi

Zeynep Nagehan Yürük Yıldırım, Alev Yılmaz, Sevinç Emre, Ilmay Bilge, Bağdagül Yavaş Aksu, Aydan Şirin

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.

Keywords: Atipik hemolitik üremik sendrom, kompleman faktör H antikorları, Ekulizumab tedavisi

References

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APA

Yürük Yıldırım, Z.N., Yılmaz, A., Emre, S., Bilge, I., Yavaş Aksu, B., & Şirin, A. (0001). Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment. Journal of Child, 17(2), 89-92. https://doi.org/10.5222/j.child.2017.089

AMA

Yürük Yıldırım Z N, Yılmaz A, Emre S, Bilge I, Yavaş Aksu B, Şirin A. Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment. Journal of Child. 0001;17(2):89-92. https://doi.org/10.5222/j.child.2017.089

ABNT

Yürük Yıldırım, Z.N.; Yılmaz, A.; Emre, S.; Bilge, I.; Yavaş Aksu, B.; Şirin, A. Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment. Journal of Child, [Publisher Location], v. 17, n. 2, p. 89-92, 0001.

Chicago: Author-Date Style

Yürük Yıldırım, Zeynep Nagehan, and Alev Yılmaz and Sevinç Emre and Ilmay Bilge and Bağdagül Yavaş Aksu and Aydan Şirin. 0001. “Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment.” Journal of Child 17, no. 2: 89-92. https://doi.org/10.5222/j.child.2017.089

Chicago: Humanities Style

Yürük Yıldırım, Zeynep Nagehan, and Alev Yılmaz and Sevinç Emre and Ilmay Bilge and Bağdagül Yavaş Aksu and Aydan Şirin. “Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment.” Journal of Child 17, no. 2 (Oct. 2024): 89-92. https://doi.org/10.5222/j.child.2017.089

Harvard: Australian Style

Yürük Yıldırım, ZN & Yılmaz, A & Emre, S & Bilge, I & Yavaş Aksu, B & Şirin, A 0001, 'Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment', Journal of Child, vol. 17, no. 2, pp. 89-92, viewed 7 Oct. 2024, https://doi.org/10.5222/j.child.2017.089

Harvard: Author-Date Style

Yürük Yıldırım, Z.N. and Yılmaz, A. and Emre, S. and Bilge, I. and Yavaş Aksu, B. and Şirin, A. (0001) ‘Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment’, Journal of Child, 17(2), pp. 89-92. https://doi.org/10.5222/j.child.2017.089 (7 Oct. 2024).

MLA

Yürük Yıldırım, Zeynep Nagehan, and Alev Yılmaz and Sevinç Emre and Ilmay Bilge and Bağdagül Yavaş Aksu and Aydan Şirin. “Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment.” Journal of Child, vol. 17, no. 2, 0001, pp. 89-92. [Database Container], https://doi.org/10.5222/j.child.2017.089

Vancouver

Yürük Yıldırım ZN, Yılmaz A, Emre S, Bilge I, Yavaş Aksu B, Şirin A. Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment. Journal of Child [Internet]. 7 Oct. 2024 [cited 7 Oct. 2024];17(2):89-92. Available from: https://doi.org/10.5222/j.child.2017.089 doi: 10.5222/j.child.2017.089

ISNAD

Yürük Yıldırım, ZeynepNagehan - Yılmaz, Alev - Emre, Sevinç - Bilge, Ilmay - Yavaş Aksu, Bağdagül - Şirin, Aydan. “Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment”. Journal of Child 17/2 (Oct. 2024): 89-92. https://doi.org/10.5222/j.child.2017.089

Volume 17, Issue 22017, P. 89-92

TIMELINE

Submitted	04.04.2017
Accepted	25.05.2017

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Case Report

Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment

Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi

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