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1

Order these events in the correct sequence of sexual differentiation:

1. Differentiation of labial scrotal folds into a scrotum

2. Genetic sex

3. Formation of seminiferous tubules

4. Formation of a testis

2. Genetic sex -->

4. Formation of a testis -->

3. Formation of seminiferous tubules -->

1. Differentiation of labial scrotal folds into a scrotum

 

2

T/F: the number of germ cells rapidly increases to a max several months after birth

F

3

T/F: There are two precursors, one precursor of the male and one precursor of the female ductal systems, that lie beside the indifferent gonads.

T

4

T/F: Germ cells migrate after the primordial gonads have developed into a testis or ovary

F

5

T/F: the indifferent bi-potential gonads have both sperm and oocytes

F

6

T/F: The ductal system develops and sends signals to the gonad to facilitate formation of either a testis or ovary

F

7

Which of the following causes the indifferent gonad to dev into an ovary?

A. Absence of testosterone

B. Presence of oocytes

C. Stimulation of early estrogen production

D. None

Which of the following causes the indifferent gonad to dev into an ovary?

A. Absence of testosterone

B. Presence of oocytes

C. Stimulation of early estrogen production

D. None

8

In a given fetus the indifferent ductal system in a 46XY fetus surprisingly develops into a uterus and vagina. Where is the MOST LIKELY defect responsible for formation of a uterus and vagina?

A. The 46XY fetus likely was missing a portion of the Y chromosome

B. The 46XY fetus could not produce testosterone

C. The 46XY fetus produced estrogen

D. The 46XY fetus produced AMH but given an estrogen milieu it was not functional

E. The 46XY fetus had a mutation in the AMH gene

In a given fetus the indifferent ductal system in a 46XY fetus surprisingly develops into a uterus and vagina. Where is the MOST LIKELY defect responsible for formation of a uterus and vagina?

A. The 46XY fetus likely was missing a portion of the Y chromosome

B. The 46XY fetus could not produce testosterone

C. The 46XY fetus produced estrogen

D. The 46XY fetus produced AMH but given an estrogen milieu it was not functional

E. The 46XY fetus had a mutation in the AMH gene

9

A 46XX fetus is surprisingly found to have a testis. Which of the following structures is the fetus also likely to have?

A. enlarged clitoris

B. hymen

C. vagina

D. seminal vesicle

E. cloaca

A 46XX fetus is surprisingly found to have a testis. Which of the following structures is the fetus also likely to have?

A. enlarged clitoris

B. hymen

C. vagina

D. seminal vesicle

E. cloaca

10

Knowing what you do about the normal HPO axis, androgen excess is most likely related to increased:

A. Granulosa cells in the ovary

B. Adrenal reticularis function

C. Pituitary release of LH

D. Pituitary release of FSH

E. Sensitivity to negative feedback

Knowing what you do about the normal HPO axis, androgen excess is most likely related to increased:

A. Granulosa cells in the ovary

B. Adrenal reticularis function

C. Pituitary release of LH (The high LH overstimulates the ovarian theca cells to make higher levels of androgens in PCOS patient.)

D. Pituitary release of FSH

E. Sensitivity to negative feedback

11

Of the following, the increased LH to FSH ratio in PCOS is most likely related to:

A. Increased sensitivity to positive feedback

B. Increased pulses of GnRH

C. Direct effect of testosterone on the pituitary

D. Increased inhibin secretion of FSH with a normal LH

Of the following, the increased LH to FSH ratio in PCOS is most likely related to: 

A. Increased sensitivity to positive feedback 

B. Increased pulses of GnRH (As GnRH (LHRH) pulse amplitude and pulse frequency are increased, the effect of this increased LHRH is an increase in secretion of LH. FSH is slightly decreased.)

C. Direct effect of testosterone on the pituitary 

D. Increased inhibin secretion of FSH with a normal LH

12

The polycystic ovaries in PCOS are likely caused by:

A. Decreased FSH

B. Increased ovarian androgens

C. Increased LH

D. Metabolic factors

E. All of the above

The polycystic ovaries in PCOS are likely caused by:

A. Decreased FSH 

B. Increased ovarian androgens 

C. Increased LH 

D. Metabolic factors 

E. All of the above

The slightly decreased FSH levels are still enough to recruit follicles (thus polycystic), but now not high enough to recruit a dominant follicle from the cohort– most of the time. The increased ovarian androgens in the ovarian microenvironment cause atrophy of follicles (thus preventing a dominant follicle as well from developing). The increased LH drives the androgen production to prevent a dominant follicle to appear. And insulin resistance seems to work directly at the ovary to accentuate androgen production (likely through the IGF receptor).

13

The abnormal bleeding in PCOS is best related to:

A. The presence of estrogen withdrawal

B. The presence of progesterone withdrawal

C. Nonfunctional feedback in H-P-O axis

D. Androgen excess

The abnormal bleeding in PCOS is best related to: 

A. The presence of estrogen withdrawal 

B. The presence of progesterone withdrawal 

C. Nonfunctional feedback in H-P-O axis 

D. Androgen excess

 

The H-P-O circuit is dysfunctional in PCOS patients preventing (most of the time) a spontaneously operative negative or positive feedback mechanism to endogenous hormones.  As a result, the patients usually don’t ovulate and have long periods of unopposed estrogen and thus endometrial proliferation. Unlike the anovulatory adolescent menstrual cycles where negative feedback is functional and allows for drops in estrogen and estrogen withdrawal, the PCOS endometrium continues to grow unabated until it is so unstable it begins to shed--- this may happen over 1 – 3 or more weeks and be excessively heavy.  They are missing the compaction and stabilization effect of progesterone that in ovulatory cycles allows a very orderly and timely shedding.  Another note:  While the HPO axis is usually not functional for feedback from endogenous levels of hormones, it does work when given pharmacologic levels of hormones (like birth control pills) to suppress gonadotropins and thus ovarian androgen production.

14

Patients with PCOS are at risk for all of the following except:

•Endometrial cancer

•Type 2 Diabetes

•Cardiovascular disease

•Unwanted pregnancy

•Pituitary adenoma

Patients with PCOS are at risk for all of the following except: 

•Endometrial cancer 

•Type 2 Diabetes 

•Cardiovascular disease 

•Unwanted pregnancy 

•Pituitary adenoma

15

A 28 year old patient is diagnosed with PCOS and is most concerned about her hirsutism. The best immediate treatment for her is: 

A. Laser hair treatment

B. Weight loss

C. Anti-androgen creams

D. Combined hormonal contraceptives

E. Anti-androgen medications

A 28 year old patient is diagnosed with PCOS and is most concerned about her hirsutism. The best immediate treatment for her is: 

A. Laser hair treatment 

B. Weight loss 

C. Anti-androgen creams 

D. Combined hormonal contraceptives 

E. Anti-androgen medications

 

These patients need suppression of their ovarian androgens first and foremost.  Combined hormonal contraceptives is the best way to begin this suppression.  It takes 4 – 6 months, however, before they will begin to notice that hair growth has slowed and new hair is not appearing. Patients are instructed that once the hormones have suppressed androgens and new hair is not appearing then they can have the hair present removed by such treatment as laser or electrolysis. Sometimes adding an anti-androgen (e.g spironolactone) adds sufficiently to the hormone therapy.  It must never be given by itself in the rare situation that if pregnancy did occur the male genitalia may be underdeveloped.  Weight loss helps at least 50% of these patients to reduce insulin resistance and break the vicious cycle that perpetuates the syndrome and they may begin to cycle on their own but this takes time and is very difficult. Finally, some but not all women find anti-androgen creams to be helpful in slowing the hair process--- but these are expensive. 

16

A patient with PCOS presents with heavy bleeding for the last  3 weeks not having had a menses in the prior 18 months.  The best immediate treatment for her is:

A. Estrogen

B. Progesterone

C. Weight loss

D. Insulin sensitizing agent

 

 

A patient with PCOS presents with heavy bleeding for the last  3 weeks not having had a menses in the prior 18 months.  The best immediate treatment for her is:

A. Estrogen 

B. Progesterone 

C. Weight loss 

D. Insulin sensitizing agent

 

These patients, when usually anovulatory, lack the stabilizing and compaction effects of progesterone.  They thus first and foremost need progesterone to stop the bleeding.  We usually given them progesterone over 2 – 3 weeks.  When we stop the progesterone, a withdrawal subsequent menses will result.  While they primarily need progesterone, if they have bled for a prolonged time, they may also need some estrogen to allow a bit of growth and “healing” effect along with the progesterone.

17

A female will achieve her maximum number of oocytes:

A. In-utero

B. By mid childhood years

C. Sometime after puberty

D. By age 40 years

E. At the menopause

A female will achieve her maximum number of oocytes:

A. In-utero 

B. By mid childhood years 

C. Sometime after puberty 

D. By age 40 years 

E. At the menopause

18

A male will achieve his maximum number of spermatocytes:

A.In-utero

B. By mid childhood years

C. Sometime after puberty

D. By age 40 years

E. After age 50 years

A male will achieve his maximum number of spermatocytes:

A.In-utero 

B. By mid childhood years 

C. Sometime after puberty 

D. By age 40 years 

E. After age 50 years

19

An adult woman is given a constant infusion of GnRH. At the conclusion of  this study blood is drawn.  Which of the following would be possible?

A. FSH levels are elevated

B. No change in FSH levels

C. FSH levels are suppressed

An adult woman is given a constant infusion of GnRH. At the conclusion of  this study blood is drawn.  Which of the following would be possible?

A. FSH levels are elevated 

B. No change in FSH levels 

C. FSH levels are suppressed

20

A 26 year old woman and her 27 year old husband  volunteer for a study and are each given an IV bolus of GnRH.  Thirty minutes later a blood sample for FSH and LH levels would find:

A. Both levels increased and  LH > FSH levels

B. Both levels increased and FSH > LH levels

C. LH and FSH levels unchanged and equivalent

D. LH and FSH levels decreased and equivalent

E. FSH levels increased and LH levels suppressed

A 26 year old woman and her 27 year old husband  volunteer for a study and are each given an IV bolus of GnRH.  Thirty minutes later a blood sample for FSH and LH levels would find:

A. Both levels increased and  LH > FSH levels 

B. Both levels increased and FSH > LH levels 

C. LH and FSH levels unchanged and equivalent 

D. LH and FSH levels decreased and equivalent 

E. FSH levels increased and LH levels suppressed

21

A 4 year old male with precocious penile development was diagnosed with an androgen producing adrenal tumor.  When compared to levels before this tumor appeared, his LH levels would most likely be: 

A. Unchanged

B. Elevated

C. Suppressed

A 4 year old male with precocious penile development was diagnosed with an androgen producing adrenal tumor.  When compared to levels before this tumor appeared, his LH levels would most likely be: 

A. Unchanged 

B. Elevated 

C. Suppressed

Too much testosterone suppresses GnRH production in the hypothalamus.

22

Which of the following best demonstrates positive feedback:

A. FSH stimulates estrogen production

B. Rising estrogen levels stimulate LH secretion

C. Decreasing estrogen levels increase FSH secretion

D. Rising estrogen levels inhibit FSH production 

Which of the following best demonstrates positive feedback:

A. FSH stimulates estrogen production (this is an example of feedforward)

B. Rising estrogen levels stimulate LH secretion 

C. Decreasing estrogen levels increase FSH secretion 

D. Rising estrogen levels inhibit FSH production 

23

A patient has radiation therapy that destroys all of her primordial follicles.  This will result in:

A. Loss of oocytes (with anovulation) but continuation of estrogen production.

B. Continued ovulation but cessation of estrogen production.

C. Loss of oocytes (with anovulation) and cessation of estrogen production.

A patient has radiation therapy that destroys all of her primordial follicles.  This will result in:

A. Loss of oocytes (with anovulation) but continuation of estrogen production. 

B. Continued ovulation but cessation of estrogen production. 

C. Loss of oocytes (with anovulation) and cessation of estrogen production.

24

A male works in a battery factory and his exposure to lead destroys his seminiferous tubules.  This will result in:

A. Loss of sperm but continuation of androgen production.

B. Continued sperm production but cessation of androgen production.

C. Loss of sperm production and cessation of androgen production. 

A male works in a battery factory and his exposure to lead destroys his seminiferous tubules.  This will result in:

A. Loss of sperm but continuation of androgen production. (unlike in the female, these functions are separate in the male)

B. Continued sperm production but cessation of androgen production. 

C. Loss of sperm production and cessation of androgen production.

25

A 27 year old woman presents with 38 day menstrual cycles and bad menstrual cramps.  She is concerned that she doesn’t ovulate because her cycles are long. Her physician explains that the menstrual cramps suggest that she in fact ovulates and that she likely ovulates on cycle day:

A. 14

B. 18

C. 20

D. 24

E. 30

A 27 year old woman presents with 38 day menstrual cycles and bad menstrual cramps.  She is concerned that she doesn’t ovulate because her cycles are long. Her physician explains that the menstrual cramps suggest that she in fact ovulates and that she likely ovulates on cycle day:

A. 14 

B. 18 

C. 20 

D. 24 

E. 30

26

A blood sample revealed FSH of 4 mIU/ml and LH of < 2 mIU/ml.  It was likely drawn from which of the following individuals?

A. 5 year old female child

B. 16 year old menstruating adolescent

C. 52 year old menopausal woman.

D.  Adult male

A blood sample revealed FSH of 4 mIU/ml and LH of < 2 mIU/ml.  It was likely drawn from which of the following individuals?

A. 5 year old female child (LHRH not being secreted)

B. 16 year old menstruating adolescent 

C. 52 year old menopausal woman. 

D.  Adult male

27

A mother brings her fraternal twins for counseling at age 13 6/12 years.  Which of them, if normally developing, is more likely capable of becoming a parent if sexually active?

A. Her daughter

B. Her son

A mother brings her fraternal twins for counseling at age 13 6/12 years.  Which of them, if normally developing, is more likely capable of becoming a parent if sexually active?

A. Her daughter 

B. Her son

28

A 6 year old male presents with Tanner  4 penile and pubic hair development.  His testes are normal for age.  Of the following, he most likely has: 

A. Hamartoma

B. Constitutional precocious puberty

C. McCune Albright syndrome

D. Testitoxicosis (LH receptor mutation in testes)

E. Adrenal testosterone secreting tumor

A 6 year old male presents with Tanner  4 penile and pubic hair development.  His testes are normal for age.  Of the following, he most likely has: 

A. Hamartoma 

B. Constitutional precocious puberty 

C. McCune Albright syndrome 

D. Testitoxicosis (LH receptor mutation in testes) 

E. Adrenal testosterone secreting tumor

29

18 month old child presents with premature breast development. She is in 75% of height as she has been since birth. She has no other symptoms. The breast development regressed after age 4 years without intervention.


A. Idiopathic central precocious puberty

B. Peripheral precocious puberty from granulosa cell tumor

C. Peripheral precocious puberty from ingestion of exogenous estrogen

D. Unusual estrogen production during infancy when FSH is usually  elevated

18 month old child presents with premature breast development. She is in 75% of height as she has been since birth. She has no other symptoms. The breast development regressed after age 4 years without intervention.


A. Idiopathic central precocious puberty

B. Peripheral precocious puberty from granulosa cell tumor

C. Peripheral precocious puberty from ingestion of exogenous estrogen

D. Unusual estrogen production during infancy when FSH is usually  elevated

30

3 year old with breast, pubic hair, and advanced growth as evidenced by growth velocity chart and bone age hand film (see next slide). What does she likely have?

A. Idiopathic central precocious puberty

B. Peripheral precocious puberty from granulosa cell tumor

C. Peripheral precocious puberty from ingestion of exogenous estrogen

D. Unusual estrogen production during infancy when FSH is usually  elevated

3 year old with breast, pubic hair, and advanced growth as evidenced by growth velocity chart and bone age hand film (see next slide). What does she likely have?


A. Idiopathic central precocious puberty

B. Peripheral precocious puberty from granulosa cell tumor (A is possible, but B is more likely)

C. Peripheral precocious puberty from ingestion of exogenous estrogen

D. Unusual estrogen production during infancy when FSH is usually  elevated