Acil Serviste Herediter Anjioödem’e Yaklaşım

Yıl 2020, Cilt: 10 Sayı: 1, 152 - 161, 16.03.2020

Öner Özdemir

https://doi.org/10.31832/smj.627591

Cited By: 1

Öz

Herediter anjioödem
(HAÖ) nadir görülen, genelde C1 inhibitör (C1-INH) eksikliğine bağlı,
tekrarlayan anjioödem ataklarıyla bilinen ölümcül olabilen otozomla dominant
geçişli bir hastalıktır. Anjioödem atakları aniden oluşur, sıklıkla tahmin
edilemez ve tipik olarak ekstremite, gövde, yüz, karın, genital organlar ve üst
solunum yollarını tutan şişliklere yol açar. Herediter anjioödem hastalarında
tipik olarak eşlik eden ürtiker olmaması sıklıkla nonhistaminerjik (bradikinerjik)
anjioödem şeklinde düşünülmesine yol açar. Ataklar önemli derecede işlevsel
bozukluğa, yaşam kalitesinin azalmasına ve larengeal atak olgularında ise ölüme
yol açabilir. Herediter anjioödemin karın tutulumuyla giden atağı genelde tanıda
gecikme ve uygunsuz tedaviye neden olan ailevi Akdeniz ateşi (FMF) ve apandisit
gibi bazı akut batın durumlarını taklit eder. Kompleksliğinden dolayı HAÖ
hastalarının bakımı bazen zorlayıcı olabilir. Herediter anjioödem tedavisi kısa
ve uzun süreli profilaksi ile atak tedavilerini içerir. Acilde HAÖ bakımı
yanlış tanı ve sınırlı sayıda tedavi seçeneği ile gecikebilir. Son iki dekattır
HAÖ atak tedavisinde bazı tedavi seçenekleri mevcuttur. Bu seçeneklerin çoğu
ABD, Avrupa, Birleşik Krallık ve Avustralya’da yaşayan HAÖ hastaları için
günümüzde ulaşılabilir durumda olmasına rağmen, gelişmekte olan ülkelerde
sınırlı sayıda tercihler hazırda bulunur. Dört tür akut/atak tedavi seçenekleri
arasında, plazma kaynaklı C1-INH konsantresi, rekombinant C1-INH konsantresi,
ecallantide ve ikatibant günümüzde kullanılanlardır.

Anahtar Kelimeler

Herediter anjioödem, atak, acil servis

Destekleyen Kurum

Yok

Proje Numarası

Yok

Teşekkür

Yok

Kaynakça

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