Flashcards in 23: Hirsutism and virilism Deck (23)
constellation of symptoms including hirsutism, deepening of the voice, frontal balding, clitoromegaly, and increased musculature.
primary causes of hirsutism and virilization include
PCOS, ovarian tumors, adrenal tumors, CAH, and Cushing syndrome
Diagnosis is made by ?
history and physical, serum assays for testosterone, DHEAS, and 17-OHP, and imaging studies.
Management involves primary treatment for the underlying cause; ?
hormonal therapy with OCPs, GnRH, or progestins; and cosmetic treatment of hirsutism.
An abnormal increase in terminal hair is due to androgen excess or increased activity of what enzyme? which converts ? to the more potent ?
testosterone to dihydrotestosterone, believed to be the main stimulant of terminal hair development.
In the adrenal glands, androgens are synthesized from the precursor ?, which is converted to ?
17α-hydroxypregnenolone-->dehydroepiandrosterone (DHEA) and its sulfate (DHEAS)-->androstenedione-->testosterone
elevated ? levels increase all the steroid hormones, including the androgens
? is derived almost entirely from the adrenal glands, so its elevation is used as a marker for adrenal androgen production.
In the ovary, any increase in ? appears to lead to excess androgen production. Further, tumors of both the ? can lead to excess androgens.
LH or in the LH:FSH ratio
adrenal gland and the ovary
Cushing syndrome may be caused by ?
do not have cushing syndrome if plasma cortisol after dexamethasone suppression tests is less than ?
pituitary adenoma, ectopic sources of ACTH, and tumors of the adrenal gland.
greater than 10 is diagnostic
Congenital adrenal hyperplasia (CAH)
most common deficiency?
-check 17-OHP: will accumulate (greater than 200 ng/dL) and is then shunted to the androgen pathway, will have salt wasting and adrenal insufficiency and ambiguous genitalia due to androgen excess
-others: 11β-hydroxylase and 3β-hydroxysteroid dehydrogenase (3β-HSD) deficiencies
confirmation test for CAH
ACTH stimulation test in which Cortrosyn (ACTH) is given IV and a 17-OHP level is checked after 1 hour. A marked increase in 17-OHP is consistent with CAH, with lower elevated values being seen in late-onset CAH and heterozygote carriers for the 21α-hydroxylase deficiency
cause of androgen excess in PCOS appears to be related to ? which are thought to be caused by an increase in the ?
excess LH stimulation leading to cystic changes in the ovaries and increased ovarian androgen secretion.
pulsatile frequency of GnRH but what causes this increased frequency is unclear.
-characterized by foci of utilization within the hyper plastic stroma
-more likely than simple hyperplasia to result in virilization as the utilized cells continue to produce ovarian androgens
Functional ovarian tumors that can produce varying amounts of androgen
sex-cord mesenchymal tumors, Sertoli-Leydig cell tumors (arrhenoblastoma), granulosa-theca cell tumors, hilar (Leydig) cell tumors, and germ cell tumors (gonadoblastomas)
a benign tumor that grows in pregnant women in response to hCG
-can result in high levels of testosterone and androstenedione and virilization in 25% of patients
-virilization of 65% of female fetuses
Androgens and corticosteroids decrease ?, leaving a greater percentage of free testosterone circulating
drugs such as ? will cause hirsutism without using androgenic pathways.
minoxidil, phenytoin, diazoxide, and cyclosporin
hirsute woman with normal free testosterone, an assay for ? is performed to determine whether increased peripheral enzymatic activity is responsible for the development of hirsutism.
Rapid onset of virilization and testosterone levels >200 ng/dL may indicate an ?
Adrenal nonneoplastic androgen suppression can be achieved with ?
glucocorticoid administration, such as prednisone 5 mg qhs. Finasteride (5α-reductase inhibitor), thus diminishes peripheral conversion of testosterone to DHT. Antiandrogens such as spironolactone have been helpful as well, but are temporizing at best.
ovarian nonneoplastic androgen production can be suppressed with ? that will suppress ? as well as increase ?
OCPs (Progesterone alone may help patients with CI to estrogen use) GnRH (requires estrogen replacement)
will suppress LH and FSH