Disorders of Puberty and Sexual Dev

Question 1

Neurokinin B _____ regulates Kisspeptin

Answer 1

Positively

Question 2

Testes Greater than ____ is puberty

Answer 2

3ccs

Question 3

What tanner stage does Male voice chaing?

Answer 3

Tanner 4

Question 4

2 Genetic causes of GnRH-dependent Precocious Puberty

Answer 4

1. Kisspeptin Receptor activation
2. NF-1 Mutation (neurofibromin)
   (SMOOTH cafe-au-lait spots, iris hamartoma, CNS tumors)

Question 5

Delayed male puberty is lack of testicular enlargement by age ____

Answer 5

• 14

Question 6

Pregnant women who have placental aromatase deficiency are unable to produce estradiol and estriol from their precursors,_____ and_____, which come from the fetus. Due to the increase in precursor levels, both mothers with placental aromatase deficiency and their female fetuses may have some signs of androgen excess

Answer 6

• DHEA sulfate

- 16-alpha-DHEA sulfate

Question 7

Kisspeptin is negatively regulated by _____ to inhibit GnRH firing

Answer 7

Dynorphin

Question 8

Name 2 GnRH INdependent Activating Receptor Mutation diseases

Answer 8

1. McCune-Albright Syndrome

2. Familial Testotoxicosis

Question 9

____ Syndrome females often have lymphedema, shield chest, and HYPERgonadotropic hypogonadism

Answer 9

Turner’s

Question 10

McCune Albright has ____ levels of FSH/LH

Answer 10

LOW

Question 11

GnRH releases in a pulsatile manner, and the GnRH neurons express _____ receptor which is believed to regulate the firing

Answer 11

KISS1

Question 12

Kallman syndrome is _____, where as Klinefelter is ____ hypogonadism

Answer 12

• HYPO

- HYPER

Question 13

Kisspeptin is positively regulated by _____ to promote GnRH firing

Answer 13

Neurokinin B

Question 14

NF-1 Precocious puberty has ____ levels of FSH/LH

Answer 14

HIGH

Question 15

in regards to GnRH, what are the 3 components to the KNDy system?

Answer 15

1. Kisspeptin
2. Neurokini B
3. Dynorphin

Question 16

How to differentiate female Tanner 3 from Tanner 4

Answer 16

• Tanner 4 has raised areola mound

Question 17

How to differentiate tanner 4 from tanner 5

Answer 17

• Tanner 4 has no pubic hair in thighs

Question 18

Key difference of Male Tanner 3 from Tanner 4

Answer 18

Tanner 5 has voice change

Question 19

McCune-Albright vs NF-1 brown spots comparison

Answer 19

• McCune has rough, irregular borders that DON’T cross the midline
• NF-1 are smooth and appear anywhere

Question 20

Dynorphin ____ regulates Kisspeptin

Answer 20

Negatively

Question 21

How many tanner stages are there?

Answer 21

5

Question 22

The onset of breast enlargement is known as ____

Answer 22

Thelarche

Question 23

____ Syndrome males often have failure of testes to grow normally, have eunuchoid proportions, and are tall. Often times diganosis is made at age 30. Look for small testes and ocassionally osteoporosis.

Answer 23

Klinefelter’s

Question 24

Aside from Brown spots in McCune-Albright, what are 2 outcomes from the over-expressed GNAS gene/G-protein signaling?

Answer 24

1. Bone dysplasia (polyostotic fibrous)

2. Sexual Precocity (GnRH-independent)

Question 25

Thelarche

Answer 25

Onset of breast enlargement

Question 26

Kisspeptin links to KISS1 receptors to regulate the ______ activity

Answer 26

GnRH Neuron

Question 27

Delayed female puberty is lack of breast development by age ____ or lack of menarche by age ____

Answer 27

• 13

- 16

Question 28

How to identify Tanner stage 2?

Answer 28

Pubic hair appears

Question 29

Familial Testotoxicosis occurs from a _____ receptor Activating mutation

Answer 29

LH