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

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

doi:10.5222/j.child.2017.089

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

Year 2017, Volume: 17 Issue: 2, 89 - 92, 01.04.2017

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

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

Abstract

Atipik hemolitik üremik sendrom aHÜS , sıklıkla son dönem böbrek yetersizliğine ilerleyen ender görülen kro- nik bir hastalıktır. Bu hastaların çoğunda alternatif komp- leman yolağının bozulmasına neden olan genetik veya edinsel bozukluk vardır. Kompleman Faktör H otoantikor- ları 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 sun- mayı amaçladık

Keywords

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

References

gasser C, gautier e, steck A, siebenmann re, Oechslin r. Hemolytic-uremic syndrome: Bilateral necrosis of the renal cortex in acute acquired hemolytic anemia. Schweiz Med Wochenschr 1955;85:905-909.
Ariceta g, Besbas n, Johnson s, Karpman D, landau D, licht C, et al. Guideline for the investiga- tion and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol 2009;24:687-96.
https://doi.org/10.1007/s00467-008-0964-1
Dragon-Durey MA, loirat C, Cloarec s, Macher MA, Blouin J, nivet H, et al. Anti-Factor H auto anti- bodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 2005;16:555-63.
https://doi.org/10.1681/ASN.2004050380
Józsi M, strobel s, Dahse HM, liu Ws, Hoyer PF, Oppermann M, et al. Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome. Blood 2007;110:1516-8.
https://doi.org/10.1182/blood-2007-02-071472
Józsi M, licht C, strobel s, Zipfel sl, richter H, Heinen s, et al. Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/ CFHR3 deficiency. Blood 2008;111:1512-4.
https://doi.org/10.1182/blood-2007-09-109876
Waters AM, licht C. aHUS caused by complement dysregulation: New therapies on the horizon. Pediatr Nephrol 2011;26:41-57.
https://doi.org/10.1007/s00467-010-1556-4
Hillmen P, Hall C, Marsh JC, elebute M, Bombara MP, Petro Be, et al. Effect of eculizumab on hemoly- sis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria. N Engl J Med 2004;350:552-9.
https://doi.org/10.1056/NEJMoa031688
loirat C, Fakhouri F, Ariceta g, Besbas n, Bitzan M, Bjerre A, et al. An international consensus appro- ach to the management of atypical hemolytic uremic syndrome in children. Pediatric Nephrol 2016;31(1):15- 39.
https://doi.org/10.1007/s00467-015-3076-8
vaisbich MH. Hemolytic-uremic syndrome in child- hood. J Bras Nefrol 2014;36:208-20.
https://doi.org/10.5935/0101-2800.20140032
sinha A, gulati A, saini s, Blanc C, gupta A, gurjar Bs, et al. Prompt plasma exchanges and immunosuppressive treatment improves the outcomes of anti-factor H autoantibody- associated hemolytic uremic syndrome in children. Kidney Int 2014;85:1151- 60.
https://doi.org/10.1038/ki.2013.373
salvadori M, Bertoni e. Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendati- ons. World J Nephrol 2013;2:56-76.
https://doi.org/10.5527/wjn.v2.i3.56
Diamante Chiodini B, Davin JC, Corazza F, Khaldi K, Dahan K, Ismaili K, et al. Eculizumab in anti- factor h antibodies associated with atypical hemolytic uremic syndrome. Pediatrics 2014;133:e1764-e1768.
https://doi.org/10.1542/peds.2013-1594
green H, Harari e, Davidovits M, Blickstein D, grossman A, gafter u, et al. Atypical HUS due to factor H antibodies in an adult patient successfully treated with eculizumab. Ren Fail 2014;36:1119-21.
https://doi.org/10.3109/0886022X.2014.917574

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

Year 2017, Volume: 17 Issue: 2, 89 - 92, 01.04.2017

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

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

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 autobodi- es have been reported in 6-11% of the patients with aHUS. We aimed to present a case with anti-FH antibody- associated 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

References

gasser C, gautier e, steck A, siebenmann re, Oechslin r. Hemolytic-uremic syndrome: Bilateral necrosis of the renal cortex in acute acquired hemolytic anemia. Schweiz Med Wochenschr 1955;85:905-909.
Ariceta g, Besbas n, Johnson s, Karpman D, landau D, licht C, et al. Guideline for the investiga- tion and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol 2009;24:687-96.
https://doi.org/10.1007/s00467-008-0964-1
Dragon-Durey MA, loirat C, Cloarec s, Macher MA, Blouin J, nivet H, et al. Anti-Factor H auto anti- bodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 2005;16:555-63.
https://doi.org/10.1681/ASN.2004050380
Józsi M, strobel s, Dahse HM, liu Ws, Hoyer PF, Oppermann M, et al. Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome. Blood 2007;110:1516-8.
https://doi.org/10.1182/blood-2007-02-071472
Józsi M, licht C, strobel s, Zipfel sl, richter H, Heinen s, et al. Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/ CFHR3 deficiency. Blood 2008;111:1512-4.
https://doi.org/10.1182/blood-2007-09-109876
Waters AM, licht C. aHUS caused by complement dysregulation: New therapies on the horizon. Pediatr Nephrol 2011;26:41-57.
https://doi.org/10.1007/s00467-010-1556-4
Hillmen P, Hall C, Marsh JC, elebute M, Bombara MP, Petro Be, et al. Effect of eculizumab on hemoly- sis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria. N Engl J Med 2004;350:552-9.
https://doi.org/10.1056/NEJMoa031688
loirat C, Fakhouri F, Ariceta g, Besbas n, Bitzan M, Bjerre A, et al. An international consensus appro- ach to the management of atypical hemolytic uremic syndrome in children. Pediatric Nephrol 2016;31(1):15- 39.
https://doi.org/10.1007/s00467-015-3076-8
vaisbich MH. Hemolytic-uremic syndrome in child- hood. J Bras Nefrol 2014;36:208-20.
https://doi.org/10.5935/0101-2800.20140032
sinha A, gulati A, saini s, Blanc C, gupta A, gurjar Bs, et al. Prompt plasma exchanges and immunosuppressive treatment improves the outcomes of anti-factor H autoantibody- associated hemolytic uremic syndrome in children. Kidney Int 2014;85:1151- 60.
https://doi.org/10.1038/ki.2013.373
salvadori M, Bertoni e. Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendati- ons. World J Nephrol 2013;2:56-76.
https://doi.org/10.5527/wjn.v2.i3.56
Diamante Chiodini B, Davin JC, Corazza F, Khaldi K, Dahan K, Ismaili K, et al. Eculizumab in anti- factor h antibodies associated with atypical hemolytic uremic syndrome. Pediatrics 2014;133:e1764-e1768.
https://doi.org/10.1542/peds.2013-1594
green H, Harari e, Davidovits M, Blickstein D, grossman A, gafter u, et al. Atypical HUS due to factor H antibodies in an adult patient successfully treated with eculizumab. Ren Fail 2014;36:1119-21.
https://doi.org/10.3109/0886022X.2014.917574

There are 25 citations in total.

Details

Primary Language	Turkish
Journal Section	Research Articles
Authors	Zeynep Yürük Yıldırım This is me Alev Yılmaz This is me Sevinç Emre This is me Ilmay Bilge This is me Bağdagül Yavaş Aksu This is me Aydan Şirin This is me
Publication Date	April 1, 2017
Published in Issue	Year 2017 Volume: 17 Issue: 2

Cite

APA	Yürük Yıldırım, Z., Yılmaz, A., Emre, S., Bilge, I., et al. (2017). Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi. Çocuk Dergisi, 17(2), 89-92. https://doi.org/10.5222/j.child.2017.089
AMA	Yürük Yıldırım Z, Yılmaz A, Emre S, Bilge I, Yavaş Aksu B, Şirin A. Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi. Çocuk Dergisi. April 2017;17(2):89-92. doi:10.5222/j.child.2017.089
Chicago	Yürük Yıldırım, Zeynep, Alev Yılmaz, Sevinç Emre, Ilmay Bilge, Bağdagül Yavaş Aksu, and Aydan Şirin. “Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom Ve Ekulizumab Tedavisi”. Çocuk Dergisi 17, no. 2 (April 2017): 89-92. https://doi.org/10.5222/j.child.2017.089.
EndNote	Yürük Yıldırım Z, Yılmaz A, Emre S, Bilge I, Yavaş Aksu B, Şirin A (April 1, 2017) Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi. Çocuk Dergisi 17 2 89–92.
IEEE	Z. Yürük Yıldırım, A. Yılmaz, S. Emre, I. Bilge, B. Yavaş Aksu, and A. Şirin, “Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi”, Çocuk Dergisi, vol. 17, no. 2, pp. 89–92, 2017, doi: 10.5222/j.child.2017.089.
ISNAD	Yürük Yıldırım, Zeynep et al. “Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom Ve Ekulizumab Tedavisi”. Çocuk Dergisi 17/2 (April 2017), 89-92. https://doi.org/10.5222/j.child.2017.089.
JAMA	Yürük Yıldırım Z, Yılmaz A, Emre S, Bilge I, Yavaş Aksu B, Şirin A. Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi. Çocuk Dergisi. 2017;17:89–92.
MLA	Yürük Yıldırım, Zeynep et al. “Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom Ve Ekulizumab Tedavisi”. Çocuk Dergisi, vol. 17, no. 2, 2017, pp. 89-92, doi:10.5222/j.child.2017.089.
Vancouver	Yürük Yıldırım Z, Yılmaz A, Emre S, Bilge I, Yavaş Aksu B, Şirin A. Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi. Çocuk Dergisi. 2017;17(2):89-92.