Premature/Delayed Puberty Flashcards Preview

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Flashcards in Premature/Delayed Puberty Deck (22):
1

GDPP - gonadotropin dependent premature puberty due to 

 

etiology

early pituitary hypothalamic gonadal axis

idiopathic

CNS lesions (harmartomas, astrocytoma, pinealoma, optic+hypothalamic glioma)

CNS irradiation

Genetics (gain of function in Kisspepten 1 and KISS-R, loss of fxn MKRN3)

Primary hypothroidism

1

responsible for facial and body hair

acne

scalp hair loss

prostate growth

DHT

2

primary hypogonadism general pathophys/etiology

 

secondary 

primary: low serum T,

high FSH+LH

 

secondary: Low serum T, low normal LH+FSH

3

typical pathophys for delayed male puberty

inadequate gonadal steroid secretion 

due to defection LH+FSH from anterior pituitary

due to defective production of GnRH from hypo+pituitary

4

secondary male hypogonadism, low T, and or sperm with low or inappropriately LH and LSH indicates _____ work-up

pituitary

5

GIPP - gonadotropin independent premature puberty due to 

 

etiology

excess secretion of sex hormones from gonads, adrenals, exogenous, ectopic production from germ cell tumor

Isosexual (leydig tumors, HcG secreting tumors, dysgerminomas)

Familial Male limted premature puberty - activating LH mutation

Adrenal path - androgen/estrogen secreting tumor, enzyme defects)

Exogenous estrogen

Pituitary gonadtotropin secreting tumomr

McCune Albright syndrome (premature+cafe au lait + bone fibrous dysplasia)

5

if inititial testing reveals GIPP (no elevation of basal LH or with GnRH) then... (testing protocol)

Testosterone, estradiol, LH, FSH, cortisol, DHEA, DHEAS 17

if hCG elevated  + tumor in mediastinum > klinefelter karyotype

testicular ultrasound

5

responsible for bone formation

breast tissue

estradiol

7

incomplete premature puberty due to

 

presentation

increased androgen production

isolated male hormone mediated sexual characterisitcs

8

McCUne albright syndrome

cause of GIPP

 

premature puberty+cafe au lait + fibrous dysplasia of bone 

9

treatment for GIPP

Ressection of adrenal or testi tumor

glucocorticoid therapy for congenital hyperplasia

anti-androgens+ aromatase inhibitor if McCune Albright of familial male limited PP

9

Klinefeltres syndrome presentation and etiology

XXY

loing bone abnl of leg independent to T deficiency
Poor insight+judgement
Impair of higher level linguistics
ADHD
Pulmonary disease
Cancer (germ cell, breast, nonhodgkins)
SLE
DM

11

Treatment GDPP

targeted toward identifiable CNS lesions

(except benign hypothalamic harmotoma)

GnRH agonist

12

if intitial testing reveals GDPP (elevated LH or LH +LSH elevation with GnRH admin) then... (testing protocol)

Pituitary MRI

estrodiol, testosterone, TSH, free T4

GH if prior cranail irradiation

13

Tanner stages

  1. prepuberty
  2. 12 - enlargement of scrotu+testes, reddening of scrotal skin+texture
  3. 13 - growth of penis (length) growth of testes and scrotum
  4. 14 - growth of penis (length+girth) darkening of scrotal skin
  5. 15 - adult size

15

premature puberty at age

9

16

exam findings indicating Klinefelter karyotyping in pt with GIPP

hCG elevated + tumor in mediastium

17

monitoring for pt being treated for premature puberty

measure LH + sex steroid prior to 2 or 3rd monthly dose (or after change)

measure bone age every 6-12 months

GnRH agonist stimulation test to test for sucess

continue until approx age 12

18

treatment familial male limited PP

spironolactone +

anti-androgen 

aromatase inhibitor (testolactone)

20

Familial male limited preamature puberty

cause of GIPP

result of activating mutation in LH receptor

21

Inititial labs and imaging for suspected premature puberty

LH levels (if elevated, GDPP)

if not elevated > stimultate with GnRH > elevated LH and FSH = GDPP)

(lack of increase = GIPP)

22

delayed male puberty = 

absence or incomplete 2ndry sex characteristic by >14y