Puberty Flashcards

1
Q

definition: adrenaarche

  • what is it responsible for?
  • when does it occur?
A
  • regeneration of zona reticularis in the adrenal cortex
  • responsible for secretion of sex steroid hormones
  • occurs between age 6-8
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2
Q

definition: gonadarche

  • what is it responsible for?
  • when does it occur?
A
  • pulsatile GnRH secretion from hypothalamus is increased
  • stimulates anterior pituitary to secrete FSH and LH
  • no phenotypic changes yet
  • begins around age 8
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3
Q

definition: thelarche

  • what is it responsible for?
  • it occurs in response to what hormone?
  • when does it occur?
A
  • breast bud development
  • first phenotypic sign of puberty
  • occurs in response to estrogen
  • occurs around age 11
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4
Q

definition: pubarche

  • when does it occur?
  • it is a result of what hormones?
  • what phase does it follow?
  • it precedes menarche by how long?
A
  • occurs around age 12
  • onset of pubic hair growth
  • likely a result of increased androgens
  • usually follow thelarche
  • precedes menarche by 2 years
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5
Q

definition: menarche

- when does it occur?

A
  • onset of menses

- average age 12-13 or 2.5 years after breast bud development

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6
Q

what happens to the HP axis during prepubertal years? why?

A

downregulation with reduction in GnRH pulses

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7
Q

what occurs with GnRH physiology at the onset of puberty?

A
  • around 8 years of age GnRH becomes enhanced during sleep
  • increased amplitude and frequency of pulsatile GnRH provoke progressively enhanced responses from pituitary of FSH and LH
  • these continue to rise throughout puberty, but LH to a larger extent
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8
Q

how / where does estrogen produce both negative and positive feedback during puberty?

A

negative - combines within inhibin to inhibit FSH response

positive - increases LH secretory response to GnRH

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9
Q

the process of sexual maturation takes how long?

A

2.5 years

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10
Q

true precocious puberty is due to what cause? how does it develop?

A

GnRH dependent sex hormone production

develops secondary to early activation of the HPG axis

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11
Q

how does GnRH precocious purberty develop?

A

results from sex hormone production of androgens and estrogens independent of HP stimulation

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12
Q

McCune Albright syndrome is characterized by what signs?

A
  • multiple bone fractures
  • cafe au lait spots
  • precocious puberty
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13
Q

what is the most common form of congenital adrenal hyperplasia? how does it present?

A

21 hydroxylase deficiency

presents with ambiguous genitalia

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14
Q

what is pathognomonic for late onset CAH?

A

elevated 17-OH progesterone

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15
Q

how does patients present with late onset CAH?

A
  • premature adrenarche
  • anovulation
  • hyperandrogenism
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16
Q

what are the main goals of treatment for precocious puberty?

A
  • arrest and diminish sexual maturation until a normal pubertal age and to maximize adult height
  • GnRH agonists are used in GnRH dependent precocious puberty
  • suppression of gonadal steroidogenesis is the treatment for GnRH independent PP
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17
Q

what is the treatment for precocious puberty?

A

GnRH agonists (ex leuprolide)

18
Q

what is the most useful test in the evaluation of premature development?

A

bone age

19
Q

what are the labs for precocious puberty?

A
  • vaginal smear
  • LH, FSH, estradiol, DHEA, TSH, 17-OHP
  • CT or MRI of brain with contrast
  • US for ovarian masses
20
Q

early puberty puts girls at risk for what condition later in life?

A

breast cancer

21
Q

when is puberty considered delayed for girls?

A
  • when secondary sex characteristics have not appeared by age 13
  • no evidence of menarche at 15-16
  • or when menses have not begun 5 years after onset of thelarche
22
Q

what is the most common cause of delayed puberty with an elevated FSH? which disease?

A

hypergonadotropic hypogonadism

turner syndrome (45 X,O)

23
Q

what is the treatment for hypergonadotropic hypogonadism?

A
  • GH early to normalize adult height

- estrogen initiated at normal time of puberty

24
Q

progestins should not be given for hypergonadotropic hypogonadism until what tanner stage?

A

IV

25
Q

definition: hypogonadotropic hypogonadism

what are the FSH and LH levels?

A
  • delay in sexual development or menses associated with low to normal levels
  • constitutional delay - most common

low to normal FSH and LH levels

26
Q

what is the most common tumor associated with delayed puberty?

A

craniopharygioma

27
Q

what are the sequalae of kallman syndrome?

A
  • olfactory tracts are hypoplastic

- arcuate nucleus of hypothalamus does not secrete GnRH (no breast development)

28
Q

what is the most common cause of primary amenorrhea in women with normal breast development?

A

mullerian agenesis

29
Q

what is mayer-rokitansky-kuster-hauser syndrome? what does it involve?

A

aka mullerian agenesis

  • congenital absence of vagina, and probably also uterine and fallopian tubes
  • ovarian function normal
30
Q

what is the simplest genital tract abnormality?

A

imperforate hymen

31
Q

what is the treatment for delayed puberty?

A
  • reassurance
  • treat underlying cause
  • possible estrogen / progesterone / GH
32
Q

tanner stage 1

A

elevation of nipple only

33
Q

tanner stage 2

A

elevation of breast and papilla as a small mound, enlargement of areolar diameter

34
Q

tanner stage 3

A

further enlargement of breast and areola with no separation of contours

35
Q

tanner stage 4

A

further enlargement with projection of areola and papilla to form a secondary mound above the level of the breast

36
Q

tanner stage 5

A

projection of papilla only, resulting from recession of the areola to the general contour of the breast

37
Q

elevation of nipple only

A

tanner stage 1

38
Q

elevation of breast and papilla as a small mound, enlargement of areolar diameter

A

tanner stage 2

39
Q

further enlargement of breast and areola with no separation of contours

A

tanner stage 3

40
Q

further enlargement with projection of areola and papilla to form a secondary mound above the level of the breast

A

tanner stage 4

41
Q

projection of papilla only, resulting from recession of the areola to the general contour of the breast

A

tanner stage 5