Çocukluk Çağında Atipik Hemolitik Üremik Sendrom

Year 2018, Volume: 45 Issue: 4, 491 - 501, 13.12.2018

Mehtap Akbalık Kara Beltinge Demircioğlu Kılıç

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

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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