Adrenogenital syndrome is a term that refers to the pronounced effect that abnormally high levels of adrenal sex hormones have on the genitalia and associated sexual characteristics.
Excess adrenal androgens secretion has masculinizing effects and is more common than the extremely rare feminizing condition seen in excess adrenal estrogen secretion.
Either of masculinizing or feminizing condition is known as adrenogenital syndrome.
SYMPTOMS OF ADRENOGENITAL SYNDROME
The symptoms that manifest in excess androgens secretion depend on:
- the sex of the individual, and
- age at which the hypersecretion first begins
In adult females…
Since androgens exert masculinizing effects, a woman suffering this disease tends to develop a male pattern of body hair, a condition termed hirsuitism.
In addition, she may also acquire other male secondary sexual characteristics such as more muscular arms and legs and deepening of voice.
Because the androgens may suppress the woman’s hypothalamo-pituitary-ovarian pathway for her own female-sex hormone secretion, the breast become smaller and menstruation usually, cease.
In newborn females…
Female infants born with adrenogenital syndrome have male-type genitalia because excessive androgen production occurs early enough during fetal life to cause the development of their genitalia along male lines, in like manner to the development of males under the influence of testicular androgen.
The clitoris, the female homolog of the male penis, grows bigger under the androgen influence and assumes a penile appearance so in some instances it is difficult at first to tell the baby’s sex.
Therefore, this hormonal disorder is one of the major causes of female pseudohermaphroditism; a condition in which ovaries (female gonads) are present but the external genitalia resembles those of a male.
(A true hermaphrodite has the gonads of both sexes)
In prepubertal males…
Excessive adrenal androgen production in prepubertal boys causes them to prematurely develop male secondary sexual characteristics such a beard, deep voice, enlarged penis, and sex drive.
This condition is called precocious pseudopuberty, the androgen released from the adrenal is not accompanied by spermatogenesis (sperm production) or any other gonadal activity, because the testes are still in their nonfunctional prepubertal state.
In adult males…
Hypersecretion of adrenal androgens in adult males has no apparent effect, because male sex characteristics already exist.
CAUSES OF ADRENOGENITAL SYNDROME
The adrenogenital syndrome is most commonly caused by an inherited enzymatic defect in the biosynthetic pathway for cortisol. The pathway for synthesis of androgens branches off the normal biosynthetic pathway for cortisol
The fall in cortisol secretion removes the negative-feedback effect on the anterior pituitary ad hypothalamus so that plasma levels of CRH and ACTH increases considerably.
The defective adrenal cortex is incapable of responding to this increased ACTH secretion with cortisol output and instead redirects more of its cholesterol precursor into the androgenic pathway. The result is excess DHEA production. This excess androgen does not inhibit ACTH secretion but rather inhibits the secretion of gonadotropins. Because gamete production is not stimulated in the absence of gonadotropins, persons with adrenogenital syndrome are sterile. Predictably, they exhibit symptoms of cortisol deficiency.