1. Adrenal steroidogenesis and congenital adrenal hyperplasia.
- Author
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Turcu AF and Auchus RJ
- Subjects
- Adrenal Cortex Neoplasms epidemiology, Adrenal Hyperplasia, Congenital drug therapy, Adrenal Hyperplasia, Congenital epidemiology, Adrenocortical Adenoma epidemiology, Aldosterone biosynthesis, Dehydroepiandrosterone biosynthesis, Dehydroepiandrosterone Sulfate metabolism, Glucocorticoids therapeutic use, Hormone Replacement Therapy, Humans, Hydrocortisone biosynthesis, Mineralocorticoids therapeutic use, Myelolipoma epidemiology, Risk Factors, Adrenal Hyperplasia, Congenital metabolism, Androgens biosynthesis, Glucocorticoids biosynthesis, Mineralocorticoids biosynthesis
- Abstract
Adrenal steroidogenesis is a dynamic process, reliant on de novo synthesis from cholesterol, under the stimulation of ACTH and other regulators. The syntheses of mineralocorticoids (primarily aldosterone), glucocorticoids (primarily cortisol), and adrenal androgens (primarily dehydroepiandrosterone and its sulfate) occur in separate adrenal cortical zones, each expressing specific enzymes. Congenital adrenal hyperplasia (CAH) encompasses a group of autosomal-recessive enzymatic defects in cortisol biosynthesis. 21-Hydroxylase (21OHD) deficiency accounts for more than 90% of CAH cases and, when milder or nonclassic forms are included, 21OHD is one of the most common genetic diseases., (Copyright © 2015 Elsevier Inc. All rights reserved.)
- Published
- 2015
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