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

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ALKAN M. , Ozterlemez N. T. , ÖZDEMİR Ç. , KURTİPEK Ö. , ARSLAN M.

GAZI MEDICAL JOURNAL, cilt.29, sa.2, ss.152-153, 2018 (ESCI İndekslerine Giren Dergi) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 29 Konu: 2
  • Basım Tarihi: 2018
  • Doi Numarası: 10.12996/gmj.2018.44
  • Sayfa Sayıları: ss.152-153


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 alpha-hydroxylase / 17,20 lyase deficiency is the least common (<1%). These enzyme deficiencies result in inadequate cortisol production and consequently increased production of corticotropin-releasing hormone (CRH) and adrenocorticotropin hormone (ACTH). Glucocorticoids and sex steroids are used for this hormonal production disorder as a long time treatment. Therefore, anesthesia management is characteristic in this patient group. We aimed to present general anesthesia management for a thyroidectomy operation planned for a 38-year-old female patient with 17 alpha-hydroxylase / 17,20 lyase deficiency with a history of steroid use.