Konjenital Hiperinsülinizmde Tanı ve Tedavi
Özet
Konjenital hiperinsülinemik hipoglisemi, pankreastan kontrolsüz insülin salınımı sonucu ortaya çıkan nadir bir hastalıktır. Hiperinsülinemik hipoglisemi (HI), plazma glikoz seviyesi düşükken uygunsuz insülin salınımı sonucu yenidoğan ve çocuklarda şiddetli ve kalıcı hipoglisemiye neden olmaktadır. HI’nin hızlı teşhis ve tedavisi hipoglisemik beyin hasarı ve uzun vadeli nörolojik komplikasyonlardan kaçınmak açısından çok önemlidir. Özellikle moleküler genetikteki gelişmeler, görüntüleme teknikler (18F-DOPA pozitron emisyon tomografisi/bilgisayarlı tomografi taraması), tıbbi tedavi ve cerrahi gelişmeler (laparoskopik ve açık pankreatektomi) HI’li hastalarda tanı ve tedavi yönetimini değiştirmiş olup prognozu iyileştirmiştir. Konjenital hiperinsülinizm erken tanı,tedavi algoritması ve tanı, tedavideki yeniliklerden bahsedilmektedir.
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