McKenna, "Dissociation of adrenal androgen
and cortisol secretion in Cushing's syndrome," Clinical Endocrinology, vol.
The clinical symptoms of CAH directly result from either the deficiencies in mineralocorticoid or glucocorticoid production or from the overproduction of adrenal androgens
. Mineralocorticoid deficiency leads to renal salt wasting, which results in severe dehydration ifleft untreated.
Although delayed pubic hair development has been observed previously, no data on adrenal androgens
have been reported in patients with IGSF1 deficiency.
DHEA is used as a marker of adrenal androgen
production; abnormally low concentrations of DHEA may occur in patients with adrenal insufficiency, older adults, and peri- and postmenopausal women.
, DHEAS, DHEA, and androstenedione, contribute to 95% of the estrogenic activity in women and are responsible for more than 50% of the androgenic activity in women.
Regardless of the patient's BMI, those who present with symptoms of PCOS, including oligo- or amenorrhea, infertility, acne, hirsutism, or ovarian cysts, should receive a thorough workup including evaluation of ovarian and adrenal androgen
hormones in addition to an assessment of insulin utility.
Some studies suggest that insulin is a negative modulator of adrenal androgen
metabolism [37, 38], although some studies using insulin sensitizers in PCOS women showed a decrease of adrenal androgens
after their administration [9, 39]; however this event could be consequent to decreases in LH levels that also occurs after metformin administration to these women .
This in turn stimulates estrogen production in the adrenal cortex or adrenal androgen
conversion to estrogen in peripheral tissue.
Dr Notelovitz pointed out that androgens may improve symptom relief in three ways: (1) by lowering sex hormone-binding globulin (SHBG) levels, which, in turn, results in a greater amount of bioavailable estrogen; (2) by a direct effect of androgens; or (3) by increased bioavailability of adrenal androgens
(low adrenal androgen
bioavailability may be induced by estrogen-alone therapy).
Prevalence of 3[beta]-hydroxysteroid dehydrogenase-deficient nonclassic adrenal hyperplasia in hyperandrogenic women with adrenal androgen
As an adrenal androgen
, DHEA acts as a steroid precursor for gonadal and peripheral testosterone and estrogen production.
These deficits trigger a compensatory increase in production of testosterone and adrenal androgens
. Correction of these aforementioned deficiencies usually reduces and normalizes the androgen excesses and associated hirsutism, although slowly.