Precocious Puberty

Question 1

definition of precocious puberty

Answer 1

premature appearance of secondary sexual characteristics;

girls <8 years

boys <9 years

Question 2

• 3. Hypothalamic (GnRH) independent (__)
• 4. True precocious puberty (__) is GnRH dependent

Answer 2

• 3. Hypothalamic (GnRH) independent (Peripheral)
• 4. True precocious puberty (Central) GnRH dependent

Question 3

variations of normal puberty

Answer 3

• 1. Premature thelarche
• 2. Premature adrenarche

Question 4

what is premature thelarche

Answer 4

breast development in girls under 3 years, due to transient ovarian cyst.

Question 5

how does bone age get affected by premature thalrche

Answer 5

bone age= chronological age. no significant advancemnet

Question 6

how is vaginal growth affected by premature thelarche

Answer 6

there is no growth of labia minora

Question 7

premature adrenarche definition

Answer 7

premature adrenal androgen secretion – usually due to pubic hair , NO CLITOROMEGALY OR breast enlargement

Question 8

in terms of physical exam how could you tell between premature adrenarche and thelarche

Answer 8

thelarche is breast development in girls <2

adrnarche is not characterized by breast development or clitoromegaly. its more about pubic hair

Question 9

outline causes of GnRH independent causes precocious puberty

Answer 9

if its gnrh independent, the problem is at the level of the gonads

Question 10

Question 11

Familial Male Limited Gonadotropin Independent Precocious Puberty

Question 12

How does bone age change in Congenital Adrenal Hyperplasia

Answer 12

• Bone age >> Chronological age

Question 13

key dermatologic feature of mcCUne Albright syndrome. What happens to sexual characteristics in this disease?

Answer 13

Cafe au lait lesions. Sexual precocity occurs.

• may have polyostotic fibrous dysplasia

Question 14

endocrinopathies associated with mcCune albright syndrome

Answer 14

pituitary adenomas

curshing syndrome

hyperthyroidism

Question 15

Causes of GnRH dependent (central) precocious puberty

Answer 15

1. idiopathic
2. CNS tumors
3. Meningitis
4. trauma
5. hydrocephalus

Question 16

Idiopathic precocious puberty– how does it affect bone age?

Answer 16

bone age >>CA.

Question 17

neurogenic precocious puberty is often because of a history of underlying __ ____.

How does it affect bone age?

Answer 17

there is a history of underlying CNS insult. BA>>>CA

Question 18

t/f in most instances of precocious puberty, bone age is > chronological age

Answer 18

true.

most of the time they are tall as children and then they have a short adult stature

Question 19

investigations to consider in a kid presenting as taller than their age and looking liek theyre going through puberty way too young.

Answer 19

Question 20

A kid presents with precocious puberty symptoms, you do a bone age and it’s normal. what do you do?

Answer 20

observe and reassess in 3-6 months. plus do other tests like adrenal androgen test, serum beta HCG, GnRH stim test etc.

Question 21

You do a gnRH stim test on a kid going through what you think is precocious puberty:

1. what if the GnRH stim test produces a blunted response (modest LH rise)?
2. what if GnRH stim test produces a significant LH rise?

Answer 21

modest/blunted LH rise: not going through actual puberty. the CNS inhibition of hypothalamic-pituitary axis is still intact and is blockin the exogenous GnRh stimulation from producing pituitary hormone.

• it is GONADOTROPIN INDEPENDENT– the problem is at the level of the pituitayr or the gonads itself.

significant LH rise: it is a central issue. it is gonadotropin dependent and a sign of “true precocious puberty”– it could be due to an organic CNS lesion or idiopathic

Question 22

general management for GnRH independent andGnRH dependent prevovious puberty

Answer 22

Question 23

Question 24

You are seeing a 16 year old boy with anosmia and
delayed puberty. You order a GnRH stimulation test
(giving iv GNRH and measuring LH and FSH). You
would expect to see:
• A. A normal rise in the LH

• B. A rise in the LH initially followed by a drop
• C. A drop in LH followed by a rise
• D. No rise in the LH

Answer 24

D. Gonadotropin deficiency. Produces dubnormal LH response to HnRH stimulation

Question 25

• You are seeing a 16 year old girl with a history
chemotherapy for treatment of leukemia as a young
child. She reports no pubertal development at all.
You would expect to see:
• A. Low estradiol levels

• B. Tanner stage 2 breast development •

C. Elevated LH and FSH

D. Both A and C

Answer 25

D– A and C

Question 26

• You are seeing a 3 year old boy with a history of
familial testotoxicosis and signs of precocious
puberty. You would expect to see:
• A. Elevated testosterone levels

• B. Tanner stage 1 pubic hair
• C. GnRH- dependent precocious puberty
• D. Both A and C

Answer 26

A.

testotoxicosis is not a central issue.– it is considered a GnRH independent precocious puberty.

Question 27

You are seeing a 4 year old girl with an onset of breast
development and pubic hair. A GnRH test shows
pubertal levels of LH and FSH. Her MRI of the head is
normal. You opt to start therapy with a GnRH analogue.
One year after therapy, you would expect:
• A. Elevated estradiol levels

• B. Progression of her bone age
• C. Flat LH and FSH after GnRH analogue therapy
• D. Both A and C

Answer 27

C. giving consistent lupron inhibits the pulsatality so reduces LH and FASH