Hellekant Puberty Flashcards

1
Q

What is adrenarche?

A

Also know as pubarche - when pubic hair growth is seen

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2
Q

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

A

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

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3
Q

What is thelarche? What hormone is responsible for it?

A

Breast development; estrogens

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4
Q

What is gonadarch?

A

Production of ovarian hormones and mature gametes

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5
Q

What is menarche?

A

Uterine responses to ovarian hormones

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6
Q

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

A

Females: 11-12; males: 13-14

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7
Q

What factors influence the age of onset for puberty?

A

Genetic, nutrions, BMI, and height

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8
Q

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

A

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

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9
Q

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

A

Gonadotropin dependent

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10
Q

What are the causes of gonadotropin dependent precocious puberty?

A

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

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11
Q

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

A

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

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12
Q

What is a common cause of GIPP in females?

A

Ovarian cysts, ovarian tumors

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13
Q

What is a common cause of GIPP in males?

A

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

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14
Q

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

A

Exogenous estrogen; adrenal pathology with excess androgen, pituitary tumors

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15
Q

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

A

GDPP; prematurely removed inhibition on HPG axis

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16
Q

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?

A

GIPP

17
Q

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

A

Elevated

18
Q

What are the levels of DHEA in GDPP?

A

Usually normal

19
Q

What labs are elevated in 21-hydroxylase def?

A

DHEA and 17OH progesterone

20
Q

What are the effects of estrogen on epiphyseal growth?

A

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

21
Q

How is the type of precocious puberty diagnosed?

A

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

22
Q

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

A

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

23
Q

What is the treatment for precocious puberty?

A

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

24
Q

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

A

GDPP

25
Q

What hormone causes the Wolffian duct to become internal genitalia?

A

Testosterone

26
Q

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

A

Anti-mullerian hormone; sertoli cells

27
Q

What enzyme is responsible for converting testosterone to dihydrotestosterone?

A

5 alpha reductase

28
Q

What hormone is responsible for the male external genitalia?

A

Dihydrotestosterone.

29
Q

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

A

17 beta estradiol

30
Q

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

A

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

31
Q

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

A

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

32
Q

When dihydrotestosterone acts on the labioscrotal folds, what forms?

A

Scrotum

33
Q

When dihydrotestosterone acts on the genital tubercle, what forms?

A

Glans penis, corpus spongiousum, corpus cavernosum

34
Q

What type of inheritance pattern is Androgen insensitivity syndrome?

A

X linked

35
Q

What happens to the Wolffian duct in complete AIS?

A

Regresses

36
Q

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

A

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