OnlineMedEd: Gynecology - "Primary Amenorrhea"

Question 1

A girl should develop secondary sex characteristics by age ______ and have her first period by ______.

Answer 1

13; 15

Question 2

What are the first two things you assess when a girl has never had a period?

Answer 2

•Does she have breast buds?
- This will tell you if her HPO axis is intact.

• Does she have a uterus?

Question 3

If a girl has breast buds and a uterus but still hasn’t bled, what three disorders could this be?

Answer 3

• Pregnancy before the first period
• Imperforate hymen
• Stress/exercise (look for the cross country runner)

Question 4

There are two disorders that present with secondary sex characteristics and an absent uterus are ______________.

Answer 4

• CAIS (XY karyotype)

* Müllerian agenesis

Question 5

There are three disorders that present with no secondary sex characteristics but a present uterus: _______________.

Answer 5

• Kallman syndrome
• Craniopharyngioma
• Turner syndrome

Question 6

How do you treat amenorrhea from a craniopharyngioma or pituitary tumor?

Answer 6

• Resect the mass

* Treat with estrogen and progesterone

Question 7

In which disorder of the HPO axis will LH and FSH be high?

Answer 7

Turner

Question 8

Without testosterone, the external genitalia become ________________.

Answer 8

female

Question 9

Why do those with CAIS not have uteruses?

Answer 9

The testes –which those with CAIS will have because testes develop from the Y chromosome’s SRY gene even in the absence of testosterone receptors –produce Müllerian inhibitory factor (MIF) which prevents the development of the uterus.

Question 10

True or false: Müllerian agenesis occurs from ectopic production of MIF.

Answer 10

False

It is idiopathic. I just made up ectopic production of MIF.

Question 11

With Müllerian agenesis and CAIS, you treat with elevating the vagina (if sex is painful). With CAIS, though, you also need to _______________.

Answer 11

remove the testes at age 21