Hellekant Puberty

Question 1

What is adrenarche?

Answer 1

Also know as pubarche - when pubic hair growth is seen

Question 2

Pubic hair growth is a result of development of what? What hormones are involved?

Answer 2

Development of the adrenal zona reticularis; production of androstenedione and dihydroepiandrosterone

Question 3

What is thelarche? What hormone is responsible for it?

Answer 3

Breast development; estrogens

Question 4

What is gonadarch?

Answer 4

Production of ovarian hormones and mature gametes

Question 5

What is menarche?

Answer 5

Uterine responses to ovarian hormones

Question 6

What age is peak height velocity seen in females? Males?

Answer 6

Females: 11-12; males: 13-14

Question 7

What factors influence the age of onset for puberty?

Answer 7

Genetic, nutrions, BMI, and height

Question 8

Precocious puberty is defined as secondary sex characteristics appearing too early; what is the cut off age in females and males?

Answer 8

Before 8 yrs old in girls, and before 9 yrs old in boys

Question 9

What type of precocious puberty is due to events that would remove the inhibition on the HP-gonadal axis?

Answer 9

Gonadotropin dependent

Question 10

What are the causes of gonadotropin dependent precocious puberty?

Answer 10

80% idiopathic; 20% CNS related: hypothalamic hamartomas, CNS tumor, cranial irradiation, hydrocephalus, trauma

Question 11

Gonadotropin independent precocious puberty is due to what type of events?

Answer 11

Events that provide peripheral hormones that carry out puberty (estrogens, androgens, ectopic tumors secreting HPG hormones)

Question 12

What is a common cause of GIPP in females?

Answer 12

Ovarian cysts, ovarian tumors

Question 13

What is a common cause of GIPP in males?

Answer 13

Leydig tumors, hCG secreting germ cell tumors (gonads, pineal, liver, retroperitoneum, posterior mediastinum tumors)

Question 14

What is a cause of GIPP in both males and females?

Answer 14

Exogenous estrogen; adrenal pathology with excess androgen, pituitary tumors

Question 15

What type of precocious puberty has all puberty signs are stage matched but occur earlier than expected?

Answer 15

GDPP; prematurely removed inhibition on HPG axis

Question 16

What type of precocious puberty is characterized by one or two signs of puberty are exhibited but not the others and those signs are staged dissimilarly?

Answer 16

GIPP

Question 17

What are the levels of DHEA in GIPP due to 21-hydroxylase insuff?

Answer 17

Elevated

Question 18

What are the levels of DHEA in GDPP?

Answer 18

Usually normal

Question 19

What labs are elevated in 21-hydroxylase def?

Answer 19

DHEA and 17OH progesterone

Question 20

What are the effects of estrogen on epiphyseal growth?

Answer 20

Biphasic: low levels of E2 favor pubertal growth spurt, high levels cause fusion of the epiphyses

Question 21

How is the type of precocious puberty diagnosed?

Answer 21

GnRH analog test: if gonadotropins increase = GDPP; if gonadotropins do not increase = GIPP

Question 22

In a random LH screening test, what level is considered pubertal? Prepubertal?

Answer 22

LH levels of 0.3 or more = pubertal; 0.1 or less is prepubertal

Question 23

What is the treatment for precocious puberty?

Answer 23

GnRH agonists (non pulsatile); GnRH antagonists; anti-estrogens; anti-androgens; if CAH need glucocorticoids

Question 24

What type of precocious puberty can result in premature epiphyseal plate closure?

Answer 24

GDPP

Question 25

What hormone causes the Wolffian duct to become internal genitalia?

Answer 25

Testosterone

Question 26

What hormone causes the the mullerian duct to degenerate? What cells secrete it?

Answer 26

Anti-mullerian hormone; sertoli cells

Question 27

What enzyme is responsible for converting testosterone to dihydrotestosterone?

Answer 27

5 alpha reductase

Question 28

What hormone is responsible for the male external genitalia?

Answer 28

Dihydrotestosterone.

Question 29

If testosterone is a substrate for aromatase, what is the end product?

Answer 29

17 beta estradiol

Question 30

When testosterone acts on the Wolffian duct, what structures form?

Answer 30

Internal genitalia of male: seminal vesicles, epididymus, vas deferens, ejaculatory duct

Question 31

When dihydrotestosterone acts on the urogenital sinus, what structures form?

Answer 31

Urinary bladder; prostatic, membranous, and proximal penile urethra; bulbourethral glads of cowper; prostate gland

Question 32

When dihydrotestosterone acts on the labioscrotal folds, what forms?

Answer 32

Scrotum

Question 33

When dihydrotestosterone acts on the genital tubercle, what forms?

Answer 33

Glans penis, corpus spongiousum, corpus cavernosum

Question 34

What type of inheritance pattern is Androgen insensitivity syndrome?

Answer 34

X linked

Question 35

What happens to the Wolffian duct in complete AIS?

Answer 35

Regresses

Question 36

What is the result of a 5 alpha reductase deficiency in an individual with XY karyotype?

Answer 36

Testicular development (with testosterone) but no DHT produced; external genitalia is female/ambiguous