Amenorrhea Flashcards
Primary amenorrhea
No menses by age 16 OR within 4 years of thelarche
Causes of primary amenorrhea
Outflow tract obstruction, end-organ disorders, central regulatory disorders
Outflow tract anomalies? How to differentiate?
Imperforate hymen- pelvic or abdominal pain from accumulation and DILATION of the vaginal vault/uterus. Bulging membrane just inside introitus with red/purple discoloration
Transverse vaginal septum- bulging septum but different from imperforate hymen bc there is a HYMENAL RING below the septum.
Vaginal agenesis- Mayer-Rokitansky-Kuster-Hauser syndrome have Mullerian agenesis or dysgenesis. Physical exam= no patent vagina, but ovaries on u/s. Neovagina by serial dilation. CAN BE CONNECTED TO UTERUS.
Testicular femininzation (AIS)- MIF is secreted bc they have testes, so no Mullerian structures. Low testosterone–> scant pubic hair but estrogen ok so breast development.
How do testicular feminization patients usually present?
No uterus so primary amenorrhea
Hypergonadotropic hypogonadism?
Low estradiol levels, with elevated FSH/LH. Primary ovarian failure causes it.
Savage syndrome
When ovaries don’t respond to FSH or LH due to RECEPTOR DEFECT.
Gonadal agenesis
Phenotypically female w/out breast development. 46XY.
Defect in enzymes involved in testicular steroid production (17-a-hydroxylase or 17,20 desmolase)
Testicular feminization
Absent or defective testosterone receptors. Phenotypically female w/out breasts.
Swyer syndrome
Congenital absence of testes in a genotypic male. No testes, no MIF. Both internal AND external female genitalia.
Hypogonadotropic hypogonadism
No GnRH so no LH or FSH. Examples:
-Kallmann syndrome.
-Compression or destruction of the pituitary stalk or arcuate nucleus.
Tumor mass effect, trauma, sarcoidosis, TB, irradiation, Hand-Schuller-Christian disease.
-Defect in GnRH pulsatility from anorexia, hyperprolactinemia, hypothyroidism, rapid weight loss, delayed puberty.
Kallman syndrome
Congenital absence of GnRH w/ anosmia.
How do you get no uterus?
Males: testes release MIF
Females: Mullerian agenesis
Treatment for absent uterus and breasts?
Estrogen replacement to get breasts and prevent osteoporosis.
Treatment for patient breasts but no uterus?
This is ok, may not req treatment
Treatment for patients with a uterus but no breasts?
Hypergonadotrophic hypogonadism likely, ERT. Further workup.
Secondary amenorrhea definition
No menses for more than 6 months or for the equivalent of 3 cycles in a woman who previously had cycles.
MCC of secondary amenorrhea
PREGNANCY
Other causes of secondary amenorrhea
Anatomic: Asherman’s, cervical stenosis
Ovarian failure: premature or menopausal
PCOS aka. Stein-Leventhal syndrome. Chronic anovulation leads to elevated levels of estrogen and androgens. The elevated androgens lead to a dec in production of SHBG, resulting in EVEN HIGHER levels of free estrogens and androgens.
Hyperprolactinemia
Rx of PCOS
Wants pregnancy: clomiphene citrate
Doesn’t want pregnancy: OCPs or progestin-only options
What stims prolactin release? What condition would create this situation?
TRH and serotonin
Hypothyroidism would cause TRH and TSH levels to spike in an effort to get the T3/T4 up.
Empty sella syndrome
What inhibits prolactin release?
Dopamine! (Dopamine agonists to treat prolactinoma, like bromocriptine or cabergoline)
What meds cause prolactin increases?
Dopamine antagonists (haldol, reglan, phenothiazine)
TCAs, MAOIs, opiates
Estrogens
Tests to rule out hyperprolactinemia
Pregnancy test, TSH, prolactin levels
Progesterone challenge test
Give to elicit withdrawal bleeding. If this happens, estrogen is present and there’s an adequate outflow tract. Amenorrhea usually therefore due to anovulation.
Progesterone challenge test doesn’t elicit bleeding. Next step?
Give estrogen & progesterone.
If STILL no bleeding, outflow tract disorder like Asherman syndrome or cervical stenosis suspected.
If bleeding results, there’s probably not enough endogenous estrogen stimulation
What next if your E&P did result in bleeding?
Figure out if it’s an HPA disorder (FSH and LH too low) or ovarian failure (which would have high levels of LH and FSH)
