Triple A sendromlu bir olgu sunumu

Yıl 2019, Cilt: 2 Sayı: 1, 34 - 37, 01.04.2019

Cevdet Duran , Zelal Adıbelli , Ufuk Kutluana Aykut Şahin Ömer Karahan

https://doi.org/10.33713/egetbd.521446

Öz

Triple A veya Allgrove sendromu, kalıtsal
geçişli bir sendrom olup, başlıca akalazya, alakrima ve adrenokortikotropin (ACTH)
direnci-addisoniyanizm ile karekterizedir. Bu yazımızda başlangıçta Addison
hastalığı tanısı konulan, ancak takiplerinde ACTH direnci-Addisoniyanizmi ve
akalazyası saptanan, akalazya için önce buji dilatasyonu, devamında Heller’s
myotomi yapılan, ayrıca yine takiplerinde alakrima ve vokal kord paralizisi
gelişen Triple A sendromlu, 48 yaşındaki bir erkek bir olguyu sunduk. Triple A
sendromunda ACTH direncine bağlı adrenal yetersizlik gelişmekte ve hastalığın diğer
komponentleri başlangıçta ortaya çıkmayabilmektedir. Ayrıca bazı olgularda
nörolojik bozukluklarda görülebilmektedir. Özellikle mineralokortikoid ihtiyacı
olmayan adrenal yetersizlikli olgular addisoniyanizm ve sendromun diğer
spesifik iki bulgusu olan akalazya ve alakrima gelişimi yönünden takip
edilmelidir. 

Anahtar Kelimeler

Triple A sendromu, Allgrove sendromu, addisoniyanizm, akalazya, alakrima

Kaynakça

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