Puberty

Question 1

Who do we eval for precocious puberty?

Answer 1

Less than 6 years old with breasts OR pubic hair, less than 8 with breasts AND pubic hair

Question 2

Types of precocious puberty?

Answer 2

GnRH-Dependent PP (early activation of HPG axis or CNS pathology) or Independent PP (ovarian tumors, McCune Albright, CAH, Adrenal disease, external exposure)

Question 3

GNRH dependent examples

Answer 3

hypopthalamic hamartomas, craniopharyngeoma, GH def, hydrocephalus, trauma, CNS infection

Question 4

GNRH independent exdysplasia of bone, amples

Answer 4

Ovarian tumor, adrenal tumor, CAH, McCune Albright

Question 5

What is McCune Albright?

Answer 5

mutation leads to activation of adenylate cyclase –> can’t stop making estrogen! cafe au lait, fibrous

Question 6

what follow up necessary with premature adrenarche

Answer 6

exclude enzyme def and adrenal mass. Serial exams/heigh check, XRAY FOR BONE AGE

Question 7

what is the most significant outcome of untreated precocious puberty?

Answer 7

short adult stature

Question 8

MOA of short stature for PP?

Answer 8

skeleton is sensitive to low E levels which leads to early epiphyseal closure

Question 9

what is a life threatening cause of precocious puberty?

Answer 9

neoplasms of CNS, ovary or adrenal

Question 10

what is included in the evaluation of precocious puberty

Answer 10

bone age, head CT to rule out brain tumor IF NEURO SXS, MRI abdomen (if high DHEA), US pelvis (ov tumor), FSH/LH/HCG, TFTs, steroids

Question 11

How to interpret bone age with PP

Answer 11

IF pubertal events and DELAYED BONE AGE, likely hypothyroidism

Question 12

what is a GnRH stim test

Answer 12

LUPRON 20 ug/kg, check LH 30-40 min later, LH > 6 = activation ofd HPG (GnRH dep)

Question 13

goals of treatment of PP

Answer 13

diagnosis life threatening disease, arrest maturation until normal pub, maximize adult height, minimize adverse psychosocial effects