021115 male puberty

Question 1

tanner stage 1

Answer 1

prepubertal

testosterone levels are relatively low

Question 2

what is considered premature male puberty in terms of age

Answer 2

under 9

Question 3

classification of premature male puberty

Answer 3

gonadotropin-dependent premature puberty (GDPP)

gonadotropin independent premature puberty (GIPP)

incomplete premature puberty (not full onset. just isolated events)

Question 4

gonadotropin-dependent premature puberty

Answer 4

due to early maturation of the hypothalamic pituitary gonadal axis

most cases are idiopathic
could be due to CNS tumor

Question 5

gonadotropin independent premature puberty

Answer 5

due to excess secretion of sex hormones derived either from gonads or adrenal glands, exogenous sources of steroids, or ectopic production of gonadotropin from a germ cell tumor

may be gender appropriate or inappropriate

Question 6

incomplete premature puberty

Answer 6

due to increased adrenal androgen production

isolated male hormone mediated sexual chracteristics (pubic/axillary hair, acne, apocrine odor)

doesn’t require therapy but should be monitored

Question 7

laboratory and imaging for premature male puberty

Answer 7

MEASURE BASAL LH LEVELS:

• if elevated, it’s GDPP
• if not elevated, stimulate w/ GnRH agonist. elevated LH and FSH means GDPP. lack of significant increase means GIPP

if it’s GDPP:

• pituitary MRI
• estradiol, testosterone, TSH, free T4
• GH if prior cranial irradiation

if GIPP:

• testosterone, estradiol, LH, FSH, cortisol, DHEA, DHEAS, 17 hydroxygprogesterone, hCG
• testicular US

Question 8

tx for premature GDPP

Answer 8

tx underlying pathology

majority of pts will need GnRH agonist (leuprolide)

Question 9

primary goal of treating premature male puberty

Answer 9

so that child can achieve normal adult height

Question 10

spironolactone

Answer 10

anti-androgen

Question 11

familial male limited premature puberty

Answer 11

activing mutation in LH receptor gene

Question 12

delayed male puberty age limit

Answer 12

over 14 years

Question 13

delayed male puberty is usually caused by

Answer 13

inadequate gonadal (testicular) steroid secretion, most often caused by defective gonadotropin secretion (LH, FSH) from pituitary due to defective production of GnRH from hypothalamus

Question 14

tx for delayed male puberty

Answer 14

watchful waiting vs androgen therapy

Question 15

causes of delayed male puberty

Answer 15

constitutional delay (fixes itself)

functional hypogonadotropic hypogonadism (illness, stress)

hypogonadotropic hypogonadism (permanent disorder)

hypergonadotropic hypogonadism

Question 16

Klinefelter’s syndrome

Answer 16

XXY

Question 17

primary vs secondary hypogonadism diagnostic work-up

Answer 17

primary-low testosterone and/or sperm with high LH and FSH. consider karyotype for Klinefelter’s syndrome

secondary-low testosterone and/or sperm with low or inappropriately normal LH and FSH. needs pituitary work up

Question 18

effects of testosterone and metabolites in males

Answer 18

see slide 50

Question 19

when evaluating for suspected hypogonadism, what should you do?

Answer 19

morning testosterone first. if normal, f/u.
if it’s low, must exclude illness, drugs, nutrition defic
repeat testosterone. and get LH, FSH. if normal, f/u.

if confirmed low testosterone, need to determine if it’s secondary or primary

Question 20

narcotics can cause secondary hypogonadism: true or false

Answer 20

true

Question 21

Kallmann syndrome

Answer 21

GnRH releasing hypothalamic neurons don’t migrate into hypothalamus