Yanık Birimlerinde Yanık Dışı Hastalar
Op. Dr. Mehmet Can1
Prof. Dr. Akgün Oral2
1Dr. Behçet Uz Çocuk Hastalıkları ve Cerrahisi EAH, Çocuk Cerrahisi Kliniği, İzmir, Türkiye
2İzmir Şehir Hastanesi, Çocuk Cerrahisi Kliniği, İzmir, Türkiye
ÖZET
Derinin konjenital veya edinsel eksfolyatif ve nekrotizan hastalıkları geniş termal yanıklarda olduğu gibi geniş cilt yüzeylerini etkileyerek benzer klinik özellikler gösterir. İlaçlara karşı gelişen bir gecikmiş tip aşırı duyarlılık reaksiyonu olan Stevens-Johnson Sendromu(SJS) ve bunun ağır formu olan Toksik Epidermal Nekroliz (TEN) yaygın purpurik döküntülerle başlayıp epidermal eksfoliasyona ilerleyen bir klinik seyir gösterir. Epidermolizis Büllosa (EB) ise çoğunlukla doğumda veya hemen sonrasında görülen epidermis veya bazal membranda de- kolman ile karakterize eksfoyatif bir hastalık grubudur. Cilt bütünlüğünde bozulmaya yol açan başka bir konjenital deri hastalığı da genellikle saçlı deride lokal deri agenezisi şeklinde ortaya çıkan Aplasia Cutis Congenita (ACC)’dır.
Etkiledikleri geniş cilt alanları nedeniyle akut dönemde termal yanıklarda olduğu gibi sıvı kaybı ve enfeksiyona zemin hazırlayan bu hastalık grubu ciddi morbidite ve mortalite riski taşırlar. Cilt dışında mukozal yüzeylerin de tutulumu da bu riski artırır. Yara enfeksiyonu, sepsis, yetersiz beslenme ve ağrı gibi sorunlar, geniş termal yanıklı hastalarda görülenlere benzerdir. Araştırmaların devam ettiği modern tedavi seçeneklerine rağmen morbidite ve mortalite oranları halen yüksektir. Geliştirilen farklı tedavide modalitelerine rağmen hiçbir tedavi yöntemi mortalite oranını azaltmada destekleyici bakımdan üstün bulunmamıştır. Hastalığın erken tanınması ve destekleyici tedavinin hızla başlanarak termal yanıklarda olduğu gibi özenli bir yara bakımı prosedürü uygulanması hayati öneme sahiptir. Bu nedenle, multidisipliner ekipleriyle yanık birimleri, bu benzersiz, kritik hasta popülasyonunun tedavi ve yönetimini sağlamak için ideal merkezlerdir.
Anahtar Kelimeler: Stevens-Johnson Sendromu; Epidermolizis Bülloza; Ekstremitelerin aplasia kutis konjenitası
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