Demystifying Adrenal Disease Douglas Stahura D.O.

Adrenal Gland Aldosterone, Cortisol and Androgens are produced in the adrenal cortex Cortex has three zones –Glomerulosa - mineralocorticoids –Fasciculata - cortisol –Reticularis - androgens

Steroid Synthesis Starting point – cholesterol Endpoint – Aldosterone, cortisol, androgens Key enzymes –17 hydroxylase –21 hydroxylase –11 hydroxylase

Steroid Synthesis

Congenital Adrenal Hyperplasia Congenital (autosomal recessive) decrease in the production of cortisol and aldosterone 95% due to 21-hydroxylase deficiency Infants/children – 21- hydroxylase deficiency is complete causing increase in DHEA and testosterone –Newborns with ambiguous genitalia

Congenital Adrenal Hyperplasia Low cortisol, because of low feedback, results in elevated ACTH = – Bilateral adrenal hyperplasia –17-OH-progesterone excess –Hirsutism in women –Precocious puberty in men