Sexual Differentation And Puberty

Question 1

In sexual differentiation, the presence of androgens is responsible for what?

Answer 1

Formation of male external genitals

Question 2

In sexual differentiation, the presence of mullerian inhibiting factor for results in what?

Answer 2

The regression a female internal duct anatomy.

Question 3

To what gender is the default pattern of differentiation?

Answer 3

Female

Question 4

The steps of male pubertal development

Answer 4

1. Testicular growth (>2.5 cm in length)
   - usually happens at 10-11 yo-14yo
2. Pubarche
3. Penile growth
4. Peak height velocity

Question 5

The steps of female pubertal development

Answer 5

1. Breast budding
   - age 8-13
2. Pubarche
3. Peak height velocity
4. Menarche

Question 6

Gynecomastia is associated with ___?

Answer 6

Klinefelter syndrome
Ketoconazole

Usually benign and asymmetric

Question 7

Galactorrhea maybe due to ___?

Answer 7

Marijuana use

Question 8

The age of delayed puberty in males and females

Treatment?

Answer 8

Males 14

Females 13

Males: refer to endo, IM testosterone qMonth

Females: refer to Endo, oral estrogen. High dose if Turner syndrome.

Question 9

Most likely cause of delayed puberty in boys

Answer 9

Constitutional delay of puberty
-bone age usually 2+ years delayed

Less likely is a tumor making HCG (this would not affect girls cuz girls need LH and FSH for puberty)

Question 10

Common causes of delayed puberty in girls

Answer 10

Constitutional delay of puberty

Functional gonadotropin deficiency (anorexia nervosa)

Primary ovarian failure (Turner syndrome)

Question 11

Premature adrenarche

Answer 11

\+androgenic characteristics
Axillary hair 
pubic hair 
acne
body odor

No estrogenic characteristics or growth spurt
Breast development
Menarche

Benign. No Tx

High DHEA and DHEA-S
Low Testosterone

Bone age is NOT advanced by >1 year

Question 12

Causes of premature adrenarche

Answer 12

Exogenous androgen
Endogenous androgen secreting tumor

Late onset CAH

Higher incidence in overweight kids (insulin resistance)

Can be an early sign of PCOS in girls

Question 13

Examples of exogenous sex steroids

Answer 13

Skin preparations

Oral contraceptives

Weightlifting steroids

Plant-based phytoestrogens

Question 14

Premature thelarche

Answer 14

<8 yo

Breast development only

Usually benign
10% get true central precocious puberty later on
–>Increased risk for PCOS

Can be due to exogenous or endogenous estrogen, or premature activation of the hypothalamic pituitary axis

Question 15

True central precocious puberty

Answer 15

<8 yo girls
<9 yo boys

Advanced bone age
Rapid linear growth crossing percentiles

The only bad thing is short adult height

Question 16

True central precocious puberty treatment

Answer 16

IM GnRH agonist (leuprolide)

Usually for boys, <6yo, very rapidly advancing bone age, psychosocial issues. Obviously need endo consult

Question 17

Finding cause of precocious puberty

Answer 17

Find cause top to bottom
-eye exam, pituitary mass?
Estrogenic if change in vaginal color or enlargement of labia minora. US may show ovarian mass.
Androgenic if muscle bulk or acne, pubic/axillary hair. US may show adrenal mass.

Question 18

Etiology of central precocious puberty seen on MRI

Answer 18

HE… MAN CHild

Hamartomas
Encephalitis

Meningitis
Arachnoid or ventricular cysts
Neoplasms

CNS trauma
Hydrocephalus

Question 19

Panhypopituitarism buzzwords

Answer 19

Micropenis
Hypoglycemia

Then…check all the pituitary hormones

Prader-Willi syndrome
Kallmann syndrome
Septo-optic dysplasia

Question 20

Androgen insensitivity syndrome

Answer 20

Genetic XY male
External female
Blind ending vagina/pouch
No uterus/ovaries (mullerian inhibiting factor is still produced)
May have inguinal masses (they are testes)
Primary amenorrhea

Due to end organ insensitivity to androgen

X-linked