190. Puberty

Question 1

Stage of pubic hair development in women:

The hair extends to the medial surface of the thighs and is distributed as an inverse triangle

Answer 1

Tanner 5

Question 2

Stage with hair spreading to the inner thighs for men

Answer 2

Tanner 5

Question 3

Breast bud develops

Answer 3

Tanner 2

Question 4

Puberty in girls:

• average pubertal onset at __ years old
• Range of normal: 8-13 years old
• Menarche average at __ years old
• Peak growth rate occurs during Tanner __-__
• Menarche: Tanner __ breast development

Answer 4

10
12.5
2-3
4

Question 5

Eating disorder that provides inadequate energy balance that suppresses production of GnRH, LH, and FSH
- no leptin activity

Answer 5

Anorexia nervosa

Question 6

Pubic hair, axillary hair, body odor, and acne are signs of __

Girls: predominantly adrenal androgens
Boys: some from gonadal testosterone

Answer 6

Adrenarche

Question 7

Stage of pubic hair development in women:

The hair spreads covering the pubis

Answer 7

Tanner 4

Question 8

Mature adult phallus and scrotum

Answer 8

Tanner 5

Question 9

Mutations in __ leads to hypogonadism then “reversal” in adulthood

Answer 9

Neurokinin B

Question 10

In addition to neuropeptides, __ __ also regulates puberty

Need to be able to provide the necessary power to fuel someone to go through puberty

Answer 10

Energy balance

Question 11

Stage of pubic hair development in women:

Sparse growth of long slightly pigmented hair straight or only slightly curly mainly along the labia

Answer 11

Tanner 2

Question 12

Delayed puberty begins at > __ years in girls and > __ in boys
- delayed puberty is also defined by an elapsed time of 4-5 years from the onset of puberty to menarche or full testicular development

Answer 12

13

15

Question 13

Mutation in Gs protein that leads to peripheral precocious puberty

Will see girls with recurring ovarian cysts

Cafe au lait spots

Fibrooptic dysplasia

Answer 13

McCune-Albright Syndrome

Question 14

Acts upstream to release kisspeptin
- controls GnRH neurons –> encodes its receptor that then release LH and FSH

Important neuropeptide for puberty, not for reproduction

Answer 14

Neurokinin B

Question 15

During infancy to late childhood/adolescence there is __ inhibition of the GnRH pulse generator
- initial “mini-puberty” after birth that lasts 6 months in boys and 12 months in girls then __ inhibition begins

Answer 15

CNS

Question 16

GnRH-dependent (from hypothalamus) triggering of early puberty

• Idiopathic (more common in girls)
• CNS abnormalities
  
  • Acquired (inflammation, surgery, trauma)
  • Congenital (hydrocephalus, hypothalamic hamartoma)
  • Tumors
  • Chronic exposure to sex steroids (CAH)

Answer 16

Central Precocious Puberty

Question 17

First signs of central puberty (Gonadarche):

• Girls: __
• Boys: __ __

Answer 17

Thelarche

Testicular enlargement

Question 18

Enlargement of breast and areola with no separation of the contours

Answer 18

Tanner 3

Question 19

Testis and scrotum enlarge further

- the penis grows mainly in length but also in breadth

Answer 19

Tanner 3

Question 20

The hair spreads covering the pubis

Answer 20

Tanner 4

Question 21

Most common mutation in Kallmann’s syndrome is in __

Answer 21

KAL1/ANOS1

Question 22

__ GnRH-producing neurons:

• Leptin
• Kiss1, GPR54
• Neurokinin B
• Adequate energy balance

Answer 22

Excitatory

Question 23

Neuropeptide that is released from adipose tissue when the body has enough body fat to provide the energy needed for puberty to take place

Answer 23

Leptin

Question 24

Scrotum, testis, and penis grow further with development of the glans and further darkening of the scrotal skin

Answer 24

Tanner 4

Question 25

Mutation in this maternally imprinted gene leads to precocious puberty Protein blocks (inhibits) the release of GnRH from the hypothalamus, thus holding off the onset of puberty

Answer 25

MKRN3

Question 26

Maturation of the hypothalamic-pituitary-adrenal axis Adrenal androgens increase - DHEA - DHEA-S - Androstenedione Independent of puberty

Answer 26

Adrenarche

Question 27

The hair is darker, coarser and curlier and spreads over the junction of the pubis

Answer 27

Tanner 3

Question 28

Gonadarche Transition period from the sexually immature state to the potentially fertile stage during which secondary sexual characteristics appear Maturation of the hypothalamic-pituitary-gonadal axis - increase in GnRH, LH, FSH, and testosterone or estrogen/estradiol

Answer 28

Puberty

Question 29

Mature breast with slightly larger nipple and recession of the areola to the general contour of the breast with projection of the papilla only

Answer 29

Tanner 5

Question 30

__ levels increase at pubertal onset | - administration of __ advances the timing of puberty

Answer 30

Kisspeptin

Question 31

Delayed puberty: __gonodatotropic = central, hypothalamic, pituitary - Acquired - autoimmune, radiation, tumor - Congenital - Kallmann, septo-optic dysplasia - Malnutrition - Anorexia - Chronic illness - Excessive exercise - Endocrinopathies - hyperprolactinemia, Cushing Syndrome

Answer 31

Hypo

Question 32

Kiss1 protein activates __ which is needed to express GnRH neurons Knockout mice have normal GnRH neurons, but gonadotropin deficiency and no puberty

Answer 32

Kiss1 receptor

Question 33

Sparse growth of pigmented hair straight or slightly curly mainly at the base of the penis

Answer 33

Tanner 2

Question 34

First menstrual period

Answer 34

Menarche

Question 35

First appearance of breast tissue

Answer 35

Thelarche

Question 36

Puberty in boys: - Average onset at __ years old - Range of normal onset: 9-15 years old - Gynecomastia: Tanner 2-3 - Voice change: Tanner __-__ - Peak growth rate: Tanner __ - Spermarche: Tanner __-__ - Facial hair: Tanner __-__

Answer 36

``` 11.5 Tanner 3-4 Tanner 4 Tanner 3-4 Tanner 4-5 ```

Question 37

Precocious puberty begins < __ years old in girls and < __ years in boys

Answer 37

8 (7 in black girls) | 9

Question 38

First appearance of pubic hair

Answer 38

Pubarche

Question 39

__ GnRH-producing neurons: - GABA - B-endorphin - MKRN3

Answer 39

Inhibitory

Question 40

GnRH-independent triggering of early puberty - Genetic (LH receptor-activating mutation, McCune-Albright Syndrome) - Tumors (adrenal, ovarian, testicular, gonadotropin producing) - Other (Hypothyroidism (Van Wyk Grumbach syndrome), exogenous sex steroids, ovarian cysts) Boys w/ penis size enlargement w/o testicle enlargement

Answer 40

Peripheral precious puberty

Question 41

Delayed puberty: __gonadotropic = primary, gonadal - Acquired - autoimmune, chemo, radiation, testicular torsion, infection (MUMPS) - Congenital - Klinefelter Syndrome (47 XXY), gonadal dysgenesis LH and FSH are high but T will be low

Answer 41

Hyper

Question 42

Late bloomers suffer from

Answer 42

Constitutional delay of puberty

Question 43

Stage of pubic hair development in women: The hair is darker, coarser and curlier and spreads over the junction of the pubis

Answer 43

Tanner 3

Question 44

The testis and scrotum enlarge and the skin of the scrotum shows some reddening and change in texture

Answer 44

Tanner 2

Question 45

Gatekeepers of puberty

Answer 45

NKB and kisspeptin

Question 46

Projection of areola and papilla to form a secondary mound above the level of the breast

Answer 46

Tanner 4

Question 47

__ begins with an increase in GnRH pulsatility and subsequent increase in gonadotropin production - increased frequency and amplitude, initially at night

Answer 47

Puberty