Amenorrhea

Question 1

Primary amenorrhea

Answer 1

No menses by age 16 OR within 4 years of thelarche

Question 2

Causes of primary amenorrhea

Answer 2

Outflow tract obstruction, end-organ disorders, central regulatory disorders

Question 3

Outflow tract anomalies? How to differentiate?

Answer 3

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.

Question 4

How do testicular feminization patients usually present?

Answer 4

No uterus so primary amenorrhea

Question 5

Hypergonadotropic hypogonadism?

Answer 5

Low estradiol levels, with elevated FSH/LH. Primary ovarian failure causes it.

Question 6

Savage syndrome

Answer 6

When ovaries don’t respond to FSH or LH due to RECEPTOR DEFECT.

Question 7

Gonadal agenesis

Answer 7

Phenotypically female w/out breast development. 46XY.

Defect in enzymes involved in testicular steroid production (17-a-hydroxylase or 17,20 desmolase)

Question 8

Testicular feminization

Answer 8

Absent or defective testosterone receptors. Phenotypically female w/out breasts.

Question 9

Swyer syndrome

Answer 9

Congenital absence of testes in a genotypic male. No testes, no MIF. Both internal AND external female genitalia.

Question 10

Hypogonadotropic hypogonadism

Answer 10

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.

Question 11

Kallman syndrome

Answer 11

Congenital absence of GnRH w/ anosmia.

Question 12

How do you get no uterus?

Answer 12

Males: testes release MIF
Females: Mullerian agenesis

Question 13

Treatment for absent uterus and breasts?

Answer 13

Estrogen replacement to get breasts and prevent osteoporosis.

Question 14

Treatment for patient breasts but no uterus?

Answer 14

This is ok, may not req treatment

Question 15

Treatment for patients with a uterus but no breasts?

Answer 15

Hypergonadotrophic hypogonadism likely, ERT. Further workup.

Question 16

Secondary amenorrhea definition

Answer 16

No menses for more than 6 months or for the equivalent of 3 cycles in a woman who previously had cycles.

Question 17

MCC of secondary amenorrhea

Answer 17

PREGNANCY

Question 18

Other causes of secondary amenorrhea

Answer 18

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

Question 19

Rx of PCOS

Answer 19

Wants pregnancy: clomiphene citrate

Doesn’t want pregnancy: OCPs or progestin-only options

Question 20

What stims prolactin release? What condition would create this situation?

Answer 20

TRH and serotonin

Hypothyroidism would cause TRH and TSH levels to spike in an effort to get the T3/T4 up.

Empty sella syndrome

Question 21

What inhibits prolactin release?

Answer 21

Dopamine! (Dopamine agonists to treat prolactinoma, like bromocriptine or cabergoline)

Question 22

What meds cause prolactin increases?

Answer 22

Dopamine antagonists (haldol, reglan, phenothiazine)

TCAs, MAOIs, opiates

Estrogens

Question 23

Tests to rule out hyperprolactinemia

Answer 23

Pregnancy test, TSH, prolactin levels

Question 24

Progesterone challenge test

Answer 24

Give to elicit withdrawal bleeding. If this happens, estrogen is present and there’s an adequate outflow tract. Amenorrhea usually therefore due to anovulation.

Question 25

Progesterone challenge test doesn’t elicit bleeding. Next step?

Answer 25

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

Question 26

What next if your E&P did result in bleeding?

Answer 26

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)