Precocious Puberty

Question 0

PPP Etiology

Answer 0

CAH
Ovarian cysts
Autonomous (McCune-Albright, testotoxicosis)
Exogenous hormones
Severe primary hypothyroidism
Adrenal/gonadal tumors

Question 1

CPP Etiology

Answer 1

Idiopathic
CNS lesions
Hypothalamic hamartoma
Tumors (neurofibroma, craniopharyngioma)
Malformation (septo-optic dysplasia, hydrocephalus, arachnoid cyst)
Infection (brain abscess, meningitis)
Trauma (surgery, irradiation, injury)

Question 2

Diagnostic clues

Answer 2

Slowly progressive/minimal changes likely benign
ANY CNS dysfunction can > CPP
Vaginal mucosa w/o estrogen: glistening red
Vaginal mucosa w/estrogen: pinkish, leukorrhea
Bone age: advanced unless changes were very rapid
CPP usually more rapid than normal puberty

Question 3

CPP Diagnosis

Answer 3

Idiopathic
CNS lesions: MRI any girl <5 and any boy
- Hypothalamic hamartoma
- Craniopharyngioma
- Malformation (septo-optic dysplasia, hydrocephalus, arachnoid cyst)
- Infection (brain abscess, meningitis)
- Trauma (surgery, irradiation, injury)
- Neurofibromatosis: pigmented lesions

Question 4

PPP Diagnosis

Answer 4

CAH: androgenic changes (body hair/odor, acne, advanced bone age) predominate
Ovarian cysts: pelvic U/S (other ovarian tumors rare, cause masculinization)
McCune-Albright: pigmented lesions, polyostotic fibrous dysplasia
Exogenous hormones: history
Severe primary hypothyroidism: TFT’s, arrested growth and other sx
Adrenal Tumors: DHEA-S extremely elevated
Gonadal Tumors: imaging
(testotoxicosis)

Question 5

CPP vs PPP

Answer 5

LH/FSH unreliable- can be normal d/t episodic secretion
GnRH Stim Test: brisk LH>FSH rise = CPP, no LH/FSH increase = immature HPG (PPP)
Estradiol, testosterone can support
CPP > testicular enlargement first

Question 6

PP Treatment

Answer 6

GnRH Agonists

GnRH pulses stimulate LH/FSH every 90-120 minutes
GnRH >/< frequent suppresses LH/FSH
Agonist binds continuously, suppressing LH/FSH

Reversible on discontinuation

Question 7

CPP Treatment

Answer 7

Resect tumors EXCEPT hypothalamic hamartoma: benign, acts as accessory hypothalamic tissue, just use GnRH agonist

Question 8

PPP Treatment

Answer 8

Treat the cause
McCune-Albright, autonomous gonadal steroid production:
- ketoconazole (blocks androgen production)
- aromatase inhibitors (block androgen > estrogen conversion)
- spironolactone (anti-androgen)