Hellekant Puberty Flashcards
What is adrenarche?
Also know as pubarche - when pubic hair growth is seen
Pubic hair growth is a result of development of what? What hormones are involved?
Development of the adrenal zona reticularis; production of androstenedione and dihydroepiandrosterone
What is thelarche? What hormone is responsible for it?
Breast development; estrogens
What is gonadarch?
Production of ovarian hormones and mature gametes
What is menarche?
Uterine responses to ovarian hormones
What age is peak height velocity seen in females? Males?
Females: 11-12; males: 13-14
What factors influence the age of onset for puberty?
Genetic, nutrions, BMI, and height
Precocious puberty is defined as secondary sex characteristics appearing too early; what is the cut off age in females and males?
Before 8 yrs old in girls, and before 9 yrs old in boys
What type of precocious puberty is due to events that would remove the inhibition on the HP-gonadal axis?
Gonadotropin dependent
What are the causes of gonadotropin dependent precocious puberty?
80% idiopathic; 20% CNS related: hypothalamic hamartomas, CNS tumor, cranial irradiation, hydrocephalus, trauma
Gonadotropin independent precocious puberty is due to what type of events?
Events that provide peripheral hormones that carry out puberty (estrogens, androgens, ectopic tumors secreting HPG hormones)
What is a common cause of GIPP in females?
Ovarian cysts, ovarian tumors
What is a common cause of GIPP in males?
Leydig tumors, hCG secreting germ cell tumors (gonads, pineal, liver, retroperitoneum, posterior mediastinum tumors)
What is a cause of GIPP in both males and females?
Exogenous estrogen; adrenal pathology with excess androgen, pituitary tumors
What type of precocious puberty has all puberty signs are stage matched but occur earlier than expected?
GDPP; prematurely removed inhibition on HPG axis
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?
GIPP
What are the levels of DHEA in GIPP due to 21-hydroxylase insuff?
Elevated
What are the levels of DHEA in GDPP?
Usually normal
What labs are elevated in 21-hydroxylase def?
DHEA and 17OH progesterone
What are the effects of estrogen on epiphyseal growth?
Biphasic: low levels of E2 favor pubertal growth spurt, high levels cause fusion of the epiphyses
How is the type of precocious puberty diagnosed?
GnRH analog test: if gonadotropins increase = GDPP; if gonadotropins do not increase = GIPP
In a random LH screening test, what level is considered pubertal? Prepubertal?
LH levels of 0.3 or more = pubertal; 0.1 or less is prepubertal
What is the treatment for precocious puberty?
GnRH agonists (non pulsatile); GnRH antagonists; anti-estrogens; anti-androgens; if CAH need glucocorticoids
What type of precocious puberty can result in premature epiphyseal plate closure?
GDPP
What hormone causes the Wolffian duct to become internal genitalia?
Testosterone
What hormone causes the the mullerian duct to degenerate? What cells secrete it?
Anti-mullerian hormone; sertoli cells
What enzyme is responsible for converting testosterone to dihydrotestosterone?
5 alpha reductase
What hormone is responsible for the male external genitalia?
Dihydrotestosterone.
If testosterone is a substrate for aromatase, what is the end product?
17 beta estradiol
When testosterone acts on the Wolffian duct, what structures form?
Internal genitalia of male: seminal vesicles, epididymus, vas deferens, ejaculatory duct
When dihydrotestosterone acts on the urogenital sinus, what structures form?
Urinary bladder; prostatic, membranous, and proximal penile urethra; bulbourethral glads of cowper; prostate gland
When dihydrotestosterone acts on the labioscrotal folds, what forms?
Scrotum
When dihydrotestosterone acts on the genital tubercle, what forms?
Glans penis, corpus spongiousum, corpus cavernosum
What type of inheritance pattern is Androgen insensitivity syndrome?
X linked
What happens to the Wolffian duct in complete AIS?
Regresses
What is the result of a 5 alpha reductase deficiency in an individual with XY karyotype?
Testicular development (with testosterone) but no DHT produced; external genitalia is female/ambiguous