BELTİNGE DEMİRCİOĞLU KILIÇ, Mehtap AKBALIK KARA

Çocukluk Çağında Atipik Hemolitik Üremik Sendrom

Atipik hemolitik üremik sendrom, alternatif komplemanın yolunun kronik kontrolsüz aktivasyonundan ortaya çıkantrombotik 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 dönemdeson 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 fonksiyonlarınınkorunması ve hematolojik remisyon sağlanması için hızla tedavi başlanmalıdır. Tanıdan sonraki ilk 24 saatte birincibasamak tedavi olarak taze donmuş plazma ile plazmaferez tedavisi başlanmalıdır, gereklilik durumunda ekuluzimabtedavisine geçilmelidir. Son dönem böbrek yetmezliği gelişmiş olgularda karaciğer veya karaciğer- böbrek nakliekuluzimab tedavisi ile birlikte planlanabilir.

Atypical Hemolytic Uremic Syndrome In Childhood

Atypical hemolytic uremic syndrome is a rare form of thrombotic microangiopathy resulting from chronic uncontrolled activation of the alternative pathway of complement. Atypical hemolytic uremic syndrome is associated with non-immune hemolytic anemia, thrombocytopenia and renal involvement. The disease causes mortality and morbidity in the acute phase and it may lead to end-stage renal failure in the long term. Although mainly kidney involvement seen in disease, extra-renal involvement can occur in 20% of cases. In diagnosed patients, genetic studies should be performed and treatment options should be performed promptly to maintain renal function and hematologic remission. Plasmapheresis treatment with fresh frozen plasma should be started as first-line therapy in the first 24 hours after diagnosis, and if necessary, treatment with eculuzimab should be started.Liver or liver-kidney transplantation with eculuzimab therapy could be planned in patients with end stage renal failure.

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