Precocious Puberty

Question 1

Compare complete and incomplete precocious puberty

Answer 1

Complete: early onset and progression of development (includes advanced bone age)

Incomplete: Isolated breast development or androgen signs

Question 2

Define central precocious puberty

Answer 2

Early activation of normal HPG axis

Question 3

Define peripheral puberty

Answer 3

Induction of puberty due to gonadal hormone production

Question 4

What is the underlying pathology in McCune Albright syndrome?

What is the classic triad?

Answer 4

• Activating mutation in G protein
• McCune Albright
  
  • Menarche comes early = precocious puberty
  • Cafe au lait spots (don’t cross midline)
  • All bones are fibrotic (Polyostotic fibrous dysplasia)

Question 5

Name 2 causes of peripheral precocious puberty specific to females and males

Answer 5

Females: ovarian cysts, granulosa cell tumor

Males: Adrenal tumor, Leydig tumor

Question 6

Define familial testotoxicosis

Answer 6

• Mutation of LH receptor -> constitutively active
• Autonomous Leydig cell activity

Tests enlarged symmetrically but less than expected for degree of virilization

Limited to boys

Question 7

How does severe primary hypothyroidism affect puberty?

Answer 7

Severe primary hypothyroidism can cause early puberty (b/c high levels of TSH act like FSH)

*But poor growth due to lack of T3/4

Question 8

Describe the laboratory evaluation of precocious puberty (2 tests)

Answer 8

1. Random serum LH and FSH
   
   • If in pubertal range, it’s central precocious puberty (Next, get an MRI)
2. If LH/FSH is low (prepubertal range), then do a GnRH stimulation test
   
   • To check if you simply missed a hormone pulse
3. If LH/FSH is still prepubertal, then it’s peripheral precocious puberty

Question 9

What is the treatment of central precocious puberty?

Answer 9

Long-acting GnRH analogue

This inhibits FSH/LH release (because it is not being released in a pulsatile manner, which would stimulate FSH/LH release)

Question 10

What is the treatment of McCune Albright?

Answer 10

Aromatase inhibitor

Question 11

What is the treatment for Congenital Adrenal Hyperplasia?

Answer 11

Glucocoticoids

(CAH = can’t synthesize glucocorticoids +- aldo)

Question 12

Define thelarche

Answer 12

Thelarche - Breast development (without other pubertal changes)

Question 13

Define adrenarche, pubarche, and gonadarche

Answer 13

• Adrenarche = Onset of adrenal androgen production
• Pubarche = Physical signs of androgens (pubic hair, axillary hair, body odor, acne)
• Gonadarche = Onset of hormone production from gonads

Question 14

Estrogen or androgens?

• Body odor?
• Axillary hair?
• Breasts?
• Pubic hair?
• Acne?

Answer 14

Body odor, pubic/axillary hair, and acne all result from androgens

Breast development is from estrogens

Question 15

Both estrogen and androgen development suggests ________ puberty in girls.

___________ suggests (similar) _____ puberty in boys.

Answer 15

Both estrogen and androgen development suggests central puberty in girls.

Teste enlargement suggests central puberty in boys.