BibTex RIS Kaynak Göster

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

Yıl 2017, Cilt: 17 Sayı: 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

Öz

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

Anahtar Kelimeler

Atipik hemolitik üremik sendrom kompleman faktör H antikorları Ekulizumab tedavisi

Kaynakça

  • 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

Yıl 2017, Cilt: 17 Sayı: 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

Öz

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

Anahtar Kelimeler

Atypical hemolytic uremic syndrome factor H autoantibodies eculizumab therapy

Kaynakça

  • 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
Toplam 25 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Bölüm Araştırma Makaleleri
Yazarlar

Zeynep Yürük Yıldırım Bu kişi benim

Alev Yılmaz Bu kişi benim

Sevinç Emre Bu kişi benim

Ilmay Bilge Bu kişi benim

Bağdagül Yavaş Aksu Bu kişi benim

Aydan Şirin Bu kişi benim

Yayımlanma Tarihi 1 Nisan 2017
Yayımlandığı Sayı Yıl 2017 Cilt: 17 Sayı: 2

Kaynak Göster

APA Yürük Yıldırım, Z., Yılmaz, A., Emre, S., Bilge, I., vd. (2017). Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi. Journal of Child, 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. Journal of Child. Nisan 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, ve Aydan Şirin. “Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom Ve Ekulizumab Tedavisi”. Journal of Child 17, sy. 2 (Nisan 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 (01 Nisan 2017) Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi. Journal of Child 17 2 89–92.
IEEE Z. Yürük Yıldırım, A. Yılmaz, S. Emre, I. Bilge, B. Yavaş Aksu, ve A. Şirin, “Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi”, Journal of Child, c. 17, sy. 2, ss. 89–92, 2017, doi: 10.5222/j.child.2017.089.
ISNAD Yürük Yıldırım, Zeynep vd. “Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom Ve Ekulizumab Tedavisi”. Journal of Child 17/2 (Nisan 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. Journal of Child. 2017;17:89–92.
MLA Yürük Yıldırım, Zeynep vd. “Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom Ve Ekulizumab Tedavisi”. Journal of Child, c. 17, sy. 2, 2017, ss. 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. Journal of Child. 2017;17(2):89-92.