what are the 2 types of hair that adults have?
vellus - non-pig’d, soft, covers entire body
terminal = pig’d, covers scalp, axilla, pubic area. Androgens stim th econversion of vellous to terminal hair.
what is an abnormal increase in terminal hair due to?
androgen excess, or increased 5alpha-reductase activity
what does 5alpha reductase do?
converts TS to DHT (more potent)
what is hirsutism?
increase in terminal hair on face, chest, back, lower abdomen, inner thighs in a woman
what is escutcheon?
shape of patch of pubic hair - diamond in a male, triangle in female
what is virilization?
dvpt of male features - voice deepening, frontal balding, increased m. mass, clitoromegaly, breast atrophy, male body habitus
what enzyme is present in z. glomerulosa but lacking in z. reticularis and z. fasciculata?
aldosterone synthase
what enzyme does the z. glomerulosa lack?
17-alpha-hydroxylase (can’t make cort & androgen)
elevations and def’ies of which hrms can cause excess androgens?
elevated ACTH
enzymatic blockade of either cort or aldo syn (stuff gets shunted towards DHEAS syn pathway)
what are the 2 categories of adrenal disorders that can cause virilization?
neoplastic (more often cause virilization)
non-neoplastic (rarely causes virilization)
what are the adrenal disorders that cause virilization?
cushing
CAH
how to make dx of Cushings?
overnight DXM suppression test - give DXM at night, then measure cort levels in a.m. Should be low in a normal pt. But if > 10microg/dL, dx of Cushings is made.
Check 24-h urine free cortisol
what is most common form of CAH?
21-alpha hydroxylase def’y
CP of CAH?
salt wasting (due to lack of aldo)
adrenal insuff’y
ambiguous genitalia at birth (due to excess androgens)
CP of “adult-onset” CAH?
mild virilization, menstrual irregularities
what labs should be checked when you suspect CAH?
17-alpha-hydroxyprogesterone (if elevated, suggests 21-alpha hydroxylase def’y)
what are the non-neoplastic ovarian disorders that can cause virilization?
PCOS
Theca Lutein cysts
stromal hyperplasia & hyperthecosis
why does androgen excess occur in PCOS?
excess LH => many ovarian cysts, increased ovarian androgen production
what is a theca lutein cyst?
theca cells that produce excess androgens that are sec’d into circl’n instead of shunting them to the granulosa cells for aromatization to estrone & estradiol
what is stromal hyperthecosis?
hyperplasia of stroma with foci of androgen production
what’s the diff b/w functional and non-functional ovarian tumors?
fct’l tumors produce androgens, nonfct’l ones do not.
what are the functional ovarian tumors?
Sertoli-Leydig cell tumors granulosa-theca cell tumors hilar (Leydig) cell tumors germ cell tumors (gonadoblastomas) luteoma (during pg'y only)
how do non-fct’l tumors cause virilization?
the stimulate the surrounding stroma => increased androgens
what labs should be ordered in w/u of hirsutism/virilization?
free TS
17-OHP
DHEAS
what does rapid-onset virilization & TS > 200 ng/dL suggest?
ovarian ca
tx of ovarian causes of hirsutism?
prednisone (suppresses androgen production) finasteride spironolactone surgery if tumor OCPs, minipill