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Journals / Dicle Tıp Dergisi / 2018 / Cilt: 45 Sayı: 4

Çocukluk Çağında Atipik Hemolitik Üremik Sendrom

Mehtap Akbalık Kara0000-0003-0790-323X
Beltinge Demircioğlu Kılıç0000-0001-9408-2139
Pages
491–501
Publication Date
2018-12-13
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Son dönem böbrek yetmezliği gelişmiş olgularda karaciğer veya karaciğer- böbrek nakli ekuluzimab tedavisi ile birlikte planlanabilir.  

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Atipik hemolitik üremik sendrom, alternatif komplemanın yolunun kronik kontrolsüz aktivasyonundan ortaya çıkan trombotik mikroanjiyopatinin nadir bir şeklidir.
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Key Findings & Data:Hastalıkta her ne kadar böbrek tutulumu görülse de, vakaların % 20'sinde ekstra-renal tutulum görülebilir.
💡
Conclusion & Impact:Son dönem böbrek yetmezliği gelişmiş olgularda karaciğer veya karaciğer- böbrek nakli ekuluzimab tedavisi ile birlikte planlanabilir.  
🔬Design:Derleme
📍Scope:Comprehensive Sample
⚙️Variables:Çocukluk çağı · atipik hemolitik üremik sendrom

Özet

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.  

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Cite This Article

Kara, M. A., Kılıç, B. D. (2018). Çocukluk Çağında Atipik Hemolitik Üremik Sendrom. Dicle Tıp Dergisi, Cilt: 45 Sayı: 4, 491–501. https://doi.org/10.5798/dicletip.497936

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