Endo - Precocious Puberty

Girls start sexual development between the ages of:

10-12

Boys start sexual development between the ages of:

12-14

Labs for adrenal gland assessment:

17-alpha- hydroxy-progesterone DHEAS

which tanner stages will Dr. Cordero test us on?

2 and 4

gonadotropin releasing hormone stimulation test procedure

2 hours in a control setting Leuprolide injection is used to provocative stimulate the pituitary gland to release the gonodotropins Gonadotropins and estradiol levels are drawn every 30 minutes

male limited precocious puberty presents at ages ____ to ____

2 to 4

prepubertal penis is ____ cm or less.

3 cm or less

pubertal penis is ____ cm or greater.

4 cm or greater

Precocious puberty in females is classified as: breast development < ___ years pubic hair before ___ years or menses before ___ years

8 (breast) 9 (pubic hair) 9 (menses)

Precocious puberty in males is classified as: testicular enlargement <___ years pubic/axillary hair before < ___ years or penile enlargement < ____ years

9 years (for all)

Aromastase Inhibitors

Anastrazole (Arimidex)

Neurogenic causes of precocious puberty

Cerebral palsy Hydrocephalus Brain irradiation/trauma Pituitary Macro/microadenomas Hamartomas

Exogenous estrogens and androgens

Contraceptive pills/creams Topical androgens: Androgel Foods injected with artificial estrogens Soy formulas Ginseng cream Hair grease/shampoo with placenta components Anabolic steroid use

Diagnostic labs (normal) for evaluation of HPG axis?

LH (0.02 -0.3) FSH (male 0.26-3.0, female 1-4.2) Estradiol (<15) Testosterone (<3 - 10)

Optic gliomas are seen in what disease?

Neurofibromatosis type 1

CONTINUE HERE

OKAY

When you don't know what you're doing what lab should you send?

TSH

Labs for hypothyroidism eval:

TSH Free T4 Thyroid peroxidase antibody TPO

After a GnRH stimulation test: LH: increased FSH: increased estradiol: increased a. central precocious puberty b. peripheral precocious puberty c. premature adrenache/thelarche

a

Caused by maternal estrogen in utero and loss of inhibition of HCG. a. premature thelarche b. premature adrenarche

a

Labs: LH: Increase FSH: increased estradiol: increased testosterone: increased what is the diagnosis? a. central precocious puberty b. adrenal cause of precocious puberty c. ovarian/testicular cause of precocious puberty d. ectopic cause of precocious puberty

a

Next step for central precocious puberty: a. brain imaging to rule out CNS tumor b. CT of abdomen and pelvis to rule out ovarian/adrenal tumors c. weight loss

a

Premature triggering of the hypothalamic pituitary-gonadal axis. a. central precocious puberty b. adrenal cause of precocious puberty c. ovarian/testicular cause of precocious puberty d. ectopic cause of precocious puberty

a

Treatment for central precocious puberty: a. gonadotropin agonist b. surgical resection & chemotherapy c. aromatase inhibitors

a

Which type of Non-Gonadotropin Dependent Precocious Pubertyis impossible to treat? a. McCune Albright Syndrome b. Testotoxicosis

a

best marker for non classical CAH a. 17-alpha- hydroxy-progesterone b. DHEAS

a

when the normal process of puberty starts too early. Most of the time there is no underlying medical cause. Less commonly, may be caused by an abnormal pituitary gland or a problem with the hypothalamus. a. central precocious puberty b. peripheral precocious puberty

a

presents with rapid onset of breast development and immediate vaginal bleeding a. ovarian tumors/cysts (granulosa cell tumor) b. adrenal carcionmas c. central cause of precocious puberty d. male limited precocious puberty (testotoxicosis)

a (cant be a central problem if it occurs this quickly)

Most common type of precocious puberty? a. central precocious puberty b. adrenal cause of precocious puberty c. ovarian/testicular cause of precocious puberty d. ectopic cause of precocious puberty

a (central 95% of the time)

increased penile gland, small testicles it is from ?

adrenal gland!

if its truly a central cause of precocious puberty, then bone age is always [delayed/advanced]

advanced

bone age in an obese child is [delayed/advanced]

advanced (they will not reach their true height)

2 drug classes used to treat Testotoxicosis:

aromatase inhibitors aldosterone antagonists

When is premature thelarche typically seen?

at birth and before 2 years of age (breast development, Dr. Cordero says by 9 months it goes away - she doesnt even do anything not even labs)

After a GnRH stimulation test: LH: decreased FSH: decreased estradiol: increased testosterone: increased a. central precocious puberty b. peripheral precocious puberty c. premature adrenache/thelarche

b

Caused by adrenal androgen production. a. premature thelarche b. premature adrenarche

b

Next step for peripheral precocious puberty: a. brain imaging to rule out CNS tumor b. CT of abdomen and pelvis to rule out ovarian/adrenal tumors c. weight loss

b

Premature adrenarche can be seen as early as what age? a. 6 months old b. 1 years old c. 2 years old d. 5 years old

b

This condition is associated with a mutation of g protein leading to precocious puberty. a. congenital hypothyroidism b. mccune albright syndrome c. congenital adrenal hyperplasia d. male limited precocious puberty (testotoxicosis)

b

Treatment for peripheral precocious puberty: a. gonadotropin agonist b. surgical resection & chemotherapy c. aromatase inhibitors

b

best marker for adrenal carcinoma: a. 17-alpha- hydroxy-progesterone b. DHEAS

b

mostly seen in overweight/obese patients a. premature thelarche b. premature adrenarche

b

occurs when the sex hormone glands (adrenal glands in boys and girls, ovaries in girls and testes in boys) begin functioning earlier than normal. a. central precocious puberty b. peripheral precocious puberty

b

osteoclasts break the bones like crazy in this syndrome a. congenital hypothyroidism b. mccune albright syndrome c. congenital adrenal hyperplasia d. male limited precocious puberty (testotoxicosis)

b

ovarian and adrenal tumors cause which type of precocious puberty? a. central precocious puberty b. peripheral precocious puberty c. Non-Gonadotropin Dependent Precocious Puberty

b

these 2 hair products can cause precocious puberty because it contains placenta oil

blue magic and cantu (mind blown, i used these when i was a child)

After a GnRH stimulation test: LH: decreased FSH: decreased estradiol: decreased testosterone: decreased a. central precocious puberty b. peripheral precocious puberty c. premature adrenache/thelarche

c

McCune Albright Syndrome and Testotoxicosis cause which type of precocious puberty? a. central precocious puberty b. peripheral precocious puberty c. Non-Gonadotropin Dependent Precocious Puberty

c

Next step for premature adrenarche/thelarche: a. brain imaging to rule out CNS tumor b. CT of abdomen and pelvis to rule out ovarian/adrenal tumors c. weight loss

c

This condition is due to a deficiency of 21 hydroxylase leading to precocious puberty. a. congenital hypothyroidism b. mccune albright syndrome c. congenital adrenal hyperplasia d. male limited precocious puberty (testotoxicosis)

c

Treatment for precocious puberty caused by testotoxicosis: a. gonadotropin agonist b. surgical resection & chemotherapy c. aromatase inhibitors

c

the backbone for androgens and estrogens?

cholesterol

most common presentation of adrenal carcinoma in a female:

clitoromegaly (always look down below!)

Patient presents with precocious sexual development early growth plates fusion short stature increased penile glands small testicles a. congenital hypothyroidism b. mccune albright syndrome c. congenital adrenal hyperplasia d. male limited precocious puberty (testotoxicosis)

congenital adrenal hyperplasia

which of the following is responsible for breast development and testicular size enlargement? a. testosterone b. cholesterol c. FSH d. estradiol

estradiol

T or F: when evaluating HPG axis in a child, all labs should be done immediately in clinic.

false (these labs need to be sen to lab corp or esoterix for accurate results)

Gold standard test to distinguish between central and peripheral precocious puberty

gonadotropin releasing hormone stimulation test

Occurs last in boys: a. penile enlargement b. testicular size enlargement c. growth spurt d. adrenarche (pubic hair)

growth spurt

[hypothyroidism/hyperthyroidism] leads to precocious puberty

hypothyroidism

Cause of central precocious puberty?

idiopathic

Once you have enlargement of the testicles then ...

it is truly precocious puberty!

treatment for premature adrenarche?

just weight loss (otherwise not tx)

oil that causes precocious puberty

lavender oil

This condition is caused by mutation of the LH receptor leading to continuous production of testosterone and therefore precocious puberty. a. congenital hypothyroidism b. mccune albright syndrome c. congenital adrenal hyperplasia d. male limited precocious puberty (testotoxicosis)

male limited precocious puberty

these patients end up having early fusion of the bone growth plates and stop growing around age 9 a. congenital hypothyroidism b. mccune albright syndrome c. congenital adrenal hyperplasia d. male limited precocious puberty (testotoxicosis)

male limited precocious puberty

Patients with this condition usually presents with cafe au lait spots on the back or front and polyostatic fibrous dysplasia a. congenital hypothyroidism b. mccune albright syndrome c. congenital adrenal hyperplasia d. male limited precocious puberty (testotoxicosis)

mccune albright syndrome

Occurs last in girls: a. adrenarche (pubic hair) b. thelarche (breast tissue) c. menarche d. growth spurt

menarche

when hypothyroid patients are treated with levothyroxine, their breast tissue will regress within [months/years}

months (3 months per the Queen)

tanner stage 1 always =

nothing (no breasts, wont be on tested on)

this enzyme can make estradiol into testosterone OR testosterone into estradiol

p450 aromatase

most common presentation of adrenal carcinomas in a males

precocious puberty

pseudo precocious puberty "triad"

premature adrenarche premature breast tissue advanced bone age

most common finding of precocious puberty associated with hypothyroidism?

premature breast development

in males with adrenal carcinomas, the testicles are [prepubertal/postpubertal]

prepubertal

Evaluation of precocious puberty include a workup for hypothyroidism and adrenal gland assessment. What 2 things are needed for the eval of precocious puberty?

prolactin level bone age (which will likely be advanced)

5 year old with advanced bone age, premature breast tissue (adipose), and premature adrenarche.. a. central precocious puberty b. pseudo precocious puberty

pseudo precocious puberty

Breasts should be examined in the [supine/prone/upright] position.

supine

adult breast a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage 5

testicular size 1- 3 a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage I

boys pubic hair: is at base of penis (1-2 slighlty pigmented) a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage II

breast budding stage contained within the areola a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage II

girls pubic hair: along the labia majora. you can count the hair - usually 1 or 2 strands. a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage II

testicular 4 mL a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage II

boys pubic hair: now into the scrotal sac. a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage III

breast tissue is no longer confined to the areola is what Dr. Cordero said (diagram says breast is more distinct but no separation between contours) a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage III

girls pubic hair: it is now impossible to count the hair. its now on the labia majora and mons. a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage III

testicular size 10, 12, or 14 a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage III

testicular size value 10-12 mL (this is what Dr. Cordero said) a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage III

boys pubic hair: more hair (everywhere) BUT not at the mid thighs yet. a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage IV

breast now has secondary mound a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage IV

girls pubic hair: is now adult type BUT is not on the inside of the thighs. a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage IV

testicular size 15-20 (this is what Dr. cordero said but it says 16 on the chart in the ppt) a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage IV

complete = what tanner stage?

tanner stage V

girls pubic hair: is now on the inside of the thighs. a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage V

testicular size 25 a. tanner stage I b. tanner stage II c. tanner stage III d. tanner stage IV e. tanner stage V

tanner stage V

Occurs first in boys: a. penile enlargement b. testicular size enlargement c. growth spurt d. adrenarche (pubic hair)

testicular size enlargement

first thing to occur in male sexual development?

testicular size enlargement

Normal sexual development sequence in boys: TAPg (boys tap girls)

testicular size enlargement adrenarche penile enlargement growth spurt

male limited precocious puberty aka

testotoxicosis

Normal sexual development sequence in girls: TAGm (girls are tagged by males)

thelarche adrenarche growth spurt menarche

Occurs first in girls: a. adrenarche (pubic hair) b. thelarche (breast tissue) c. menarche d. growth spurt

thelarche (breast tissue)

T or F: Premarin if used for more than 3 weeks can lead to precocious puberty

true

T or F: premature adrenarche is benign/normal variant of precocious puberty.

true

T or F: premature thelarche is benign/normal variant of precocious puberty.

true

premature adrenarche is benign

true (Dr. Cordero tells the mama to come back in 6 months)

Gonadotropin agonists

◦Supprelin Implant (Histrelin Acetate) ◦Lupron Depo Injection ◦Triptodur (Triptorelin)◦Supprelin Implant (Histrelin Acetate) ◦Lupron Depo Injection ◦Triptodur (Triptorelin)