Metin ALKAN, Ömer KURTİPEK, Naciye TÜRK ÖZTERLEMEZ, Çağrı ÖZDEMİR, Mustafa ARSLAN

17 Αlfa Hidroksilaz/17,20 Liyaz Eksikliği olan Hastada Anestezi Yönetimi

Konjenital adrenal hiperplazi (KAH), adrenal kortekste kortizol ve seks steroidlerin biyosentezinde yer alan enzimlerden herhangi birinin kalıtsal kusurundan kaynaklanan otozomal resesif bozukluktur. En sık 21-hidroksilaz eksikliği görülmesine rağmen 17 ?-hidroksilaz/17,20 liyaz eksikliği en az görülen tipidir (

Anesthesia Management in a Patient with 17 Αlpha Hydroxylase/17,20 Lyase Deficiency

Congenital adrenal hyperplasia (CAH) is a an autosomal recessive disorder resulting from a defect in any of several enzymes involved in biosenthesis of cortisol and sex steroids in adrenal cortex. 21-hydroxylase deficiency (90-95%) is the most common form and 17 α-hydroxylase / 17,20 lyase deficiency is the least common (

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