Reproduction and Hypogonadism

Question 1

What is the function of testosterone in the Leydig cells?

Answer 1

Testosterone maintains Wolffian/Male structures.

Question 2

What is the result in the absence of SRY, testosterone and Mullein Inhibiting Factor (MIF)?

Answer 2

Female development

Question 3

What is the most common cause of ambiguous genitalia in 46 XX female?

Answer 3

Congenital Adrenal Hyperplasia

Question 4

A neonate presents in acute adrenal crisis and with ambiguous genitalia. What do you do?

Answer 4

This is likely CAH. Give normal saline and IV hydrocortisone.

Question 5

A 17year old girl presents with 3 months of amenorrhea. Since menarche at age 12, her menses have been irregular, with episodes of amenorrhea lasting as long as 3 mos. In the past, oral PG every 3mos resulted in a bleed. She took a course of PG last week but did not bleed.
Exam shows a BMI of 25, some breast engorgement is noted but no galactorrhea. Pelvic exam is normal.
Labs show: PRL 45 (10-22), fT4 15 (10-25)

What is the most appropriate first step in managing this patient?

a. MRI of sella
b. prescribe bromocriptine
c. order TSH
d. order beta-HCG
e. order FSH

Answer 5

d. order beta-HCG

Question 6

What is the differential diagnosis for primary amenorrhea?

Answer 6

Things that can cause secondary amenorrhea = hypothalamic, ant. pit., PCOS, POF, pregnancy, hyperprolactinemia, thyroid, outflow tract.

Also: outflow tract blockage, failure of mullein duct development, genetic abnormalities (Fragile X, Turner’s), male genotype with androgen receptor resistance, late-onset congenital adrenal hyperplasia.

Question 7

A 31year old woman presents with 6 mos of amenorrhea. She has had 3 previous pregnancies. A pregnancy test is negative now. She has no withdrawal bleed to PG that was given for 10days. Her exam (including a pelvic exam) is normal. What would be your next step?
A. OrdersellaMRI
B. Order pelvic US
C. Order an estradiol level
D. Order FSH level
E. Prescribe the OCP

Answer 7

d. order FSH level

Question 8

Hypergonadotropic hypogonadism is caused by:

a. cranopharyngioma
b. prolactinoma
c. anorexia nervosa
d. autoimmune oopheritis
e. chronic renal failure

Answer 8

d. autoimmune oopheritis; we are looking for the primary cause in this answer set.

Question 9

PCOS is characterized by all the following EXCEPT:
A) Hirsutism and menstrual disorder typically have
their onset at puberty
B) Thecourseofhirsutismandmenstrualdisorder is characterized by gradual onset and slow progression
C) Serum testosterone is markedly elevated to a level 2-3x normal
D) High PRL may be present in 20% of patients
E) The hyperandrogenicity is LH-dependent and usually responds to LH-suppressive therapy such as oral contraceptive pill therapy

Answer 9

C) Serum testosterone is markedly elevated to a level 2-3x normal. The serum testosterone could be high but it doesn’t have to be high.

Question 10

What can mimic PCOS?

Answer 10

Adult onset CAH (looks pretty much the same!), Cushing’s syndrome, androgen secreting tumours (adrenal and ovarian)

Question 11

What are patients with PCOS at risk for?

Answer 11

Infertility
Metabolic syndrome: DM, HTN, dyslipidemia
Gestational DM
Endometrial cancer (if estrogen is unopposed)

Question 12

All of the following would directly result in an
increased serum PRL level EXCEPT: A. Pregnancy
B. Renal Failure
C. Hypothyroidism
D. Heart Failure
E. Sarcoidosis

Answer 12

d. heart failure

Question 13

A healthy 23year old woman has amenorrhea and galactorrhea of 2 years’ duration. Serum PRL is 280ng/mL (0-23). The most likely cause is:
A) Primary hypothyroidism
B) Use of dopaminergic drugs
C) Chronic renal failure
D) Suprasellar craniopharyngioma
E) A prolactin producing pituitary tumor

Answer 13

e. prolactin producing pituitary tumour

Question 14

You image this patient’s sella and the MRI shows a 5mm pituitary tumor (PRL 280 (Normal 0-23). What is your diagnosis?
A) Macroprolactinoma
b) Pseudoprolactinoma
c) Microprolactinoma

Answer 14

c. microprolactinemia (

Question 15

Consider instead that you image this patient’s sella and see a 3cm pituitary tumor with suprasellar extension (PRL 100 (Normal 0-23), what is your diagnosis?
A) Macroprolactinoma
b) Pseudoprolactinoma
c) Microprolactinoma

Answer 15

b) Pseudoprolactinoma

Question 16

Now Consider instead that you have a patient with a prolactin level of 1000, and you image this patient’s sella and see a 3cm pituitary tumor with suprasellar extension, what is your diagnosis?
A) Macroprolactinoma
b) Pseudoprolactinoma
c) Microprolactinoma

Answer 16

A) Macroprolactinoma

Question 17

Primary amenorrhea may occur in association with:
A) Failure of mullerian duct development
B) Testicular feminization syndrome ( a genetic male with androgen insensitivity)
C) Late-onset congenital adrenal hyperplasia due to 21-hydroxylase deficiency
D) Craniopharyngioma
E) All of the above

Answer 17

e) all of the above

Question 18

Which statement is true regarding Kallmann’s syndrome;
A) Hypogonadotropic hypogonadism due to absence of GnRH-producing cells in the hypothalamus
B) Gonadotropin deficiency is familial and may be associated with other anterior pituitary hormone deficiency
C) The testes show hyalinization of the seminiferous tubules, and there is irreversible azoospermia
D) A hypothalamic mass is usually seen on head MRI
E) The karyotype is 46XX

Answer 18

A

Question 19

Presence of hypergonadotropic hypogonadism in a pubertal male may be associated with all the following except
A) Failure of sexual maturation
B) Eunuchoidalbodyproportions
C) Premature closure of the epiphyses and permanent shortness of stature
D) Gynecomastia
E) Absence of rise in testosterone levels with HCG therapy

Answer 19

c)