Çocukluk Çağında Atipik Hemolitik Üremik Sendrom

Mehtap Akbalık Kara; Beltinge Demircioğlu Kılıç

doi:10.5798/dicletip.497936

Çocukluk Çağında Atipik Hemolitik Üremik Sendrom

Abstract

Atipik hemolitik üremik sendrom, alternatif komplemanın yolunun kronik kontrolsüz aktivasyonundan ortaya çıkan trombotik mikroanjiyopatinin nadir bir şeklidir. Atipik hemolitik üremik sendrom, immün olmayan hemolitik anemi, trombositopeni ve böbrek tutulumu ile ilişkilidir. Hastalık akut dönemde mortalite ve morbiditeye ve uzun dönemde son dönem böbrek yetmezliğine neden olabilir. Hastalıkta her ne kadar böbrek tutulumu görülse de, vakaların % 20'sinde ekstra-renal tutulum görülebilir. Tanı konan hastalarda genetik çalışma yapılmalı böbrek fonksiyonlarının korunması ve hematolojik remisyon sağlanması için hızla tedavi başlanmalıdır. Tanıdan sonraki ilk 24 saatte birinci basamak tedavi olarak taze donmuş plazma ile plazmaferez tedavisi başlanmalıdır, gereklilik durumunda ekuluzimab tedavisine geçilmelidir. Son dönem böbrek yetmezliği gelişmiş olgularda karaciğer veya karaciğer- böbrek nakli ekuluzimab tedavisi ile birlikte planlanabilir.

Keywords

Çocukluk çağı,atipik hemolitik üremik sendrom

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Details

Primary Language

Turkish

Subjects

-

Journal Section

Review

Authors

Mehtap Akbalık Kara ^* This is me
0000-0003-0790-323X
Türkiye

Beltinge Demircioğlu Kılıç This is me
0000-0001-9408-2139
Türkiye

Publication Date

December 13, 2018

Submission Date

December 16, 2018

Acceptance Date

October 8, 2018

Published in Issue

Year 2018 Volume: 45 Number: 4

DOI

https://doi.org/10.5798/dicletip.497936

IZ

https://izlik.org/JA69FE39KS

Cite

RIS / Bibtex

APA

Kara, M. A., & Demircioğlu Kılıç, B. (2018). Çocukluk Çağında Atipik Hemolitik Üremik Sendrom. Dicle Medical Journal, 45(4), 491-501. https://doi.org/10.5798/dicletip.497936

AMA

1.Kara MA, Demircioğlu Kılıç B. Çocukluk Çağında Atipik Hemolitik Üremik Sendrom. Dicle Medical Journal. 2018;45(4):491-501. doi:10.5798/dicletip.497936

Chicago

Kara, Mehtap Akbalık, and Beltinge Demircioğlu Kılıç. 2018. “Çocukluk Çağında Atipik Hemolitik Üremik Sendrom”. Dicle Medical Journal 45 (4): 491-501. https://doi.org/10.5798/dicletip.497936.

EndNote

Kara MA, Demircioğlu Kılıç B (December 1, 2018) Çocukluk Çağında Atipik Hemolitik Üremik Sendrom. Dicle Medical Journal 45 4 491–501.

IEEE

[1]M. A. Kara and B. Demircioğlu Kılıç, “Çocukluk Çağında Atipik Hemolitik Üremik Sendrom”, Dicle Medical Journal, vol. 45, no. 4, pp. 491–501, Dec. 2018, doi: 10.5798/dicletip.497936.

ISNAD

Kara, Mehtap Akbalık - Demircioğlu Kılıç, Beltinge. “Çocukluk Çağında Atipik Hemolitik Üremik Sendrom”. Dicle Medical Journal 45/4 (December 1, 2018): 491-501. https://doi.org/10.5798/dicletip.497936.

JAMA

1.Kara MA, Demircioğlu Kılıç B. Çocukluk Çağında Atipik Hemolitik Üremik Sendrom. Dicle Medical Journal. 2018;45:491–501.

MLA

Kara, Mehtap Akbalık, and Beltinge Demircioğlu Kılıç. “Çocukluk Çağında Atipik Hemolitik Üremik Sendrom”. Dicle Medical Journal, vol. 45, no. 4, Dec. 2018, pp. 491-0, doi:10.5798/dicletip.497936.

Vancouver

1.Mehtap Akbalık Kara, Beltinge Demircioğlu Kılıç. Çocukluk Çağında Atipik Hemolitik Üremik Sendrom. Dicle Medical Journal. 2018 Dec. 1;45(4):491-50. doi:10.5798/dicletip.497936