Puberty Flashcards

1
Q

What are the three levels of differences between males and females?

A

genetic sex

gonadal sex

phenotypic sex

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2
Q

This definition of sex…

apparent anatomic sex of an individual

determined by gonadal sex

determined by presence or absence of masculizing hormones

A

phenotypic sex

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3
Q

This definition of sex…

determined by development of testes or ovaries

Determined by presence or absence of SRY gene

presence or absence of Y chromosome

A

Gonadal sex

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4
Q

This definition of sex…

depends on combination of sex chromosomes at time of conception

A

genetic sex

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5
Q

The SRY gene makes what substance, which promotes…

A

Testes determining factor

promotes testis differentiation

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6
Q

Do males or females lack the SRY gene?

A

females

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7
Q

The absence of SRY in females means gonads don’t receive signal for…

A

testicular formation

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8
Q

What is required for the active genetic pathways necessary for ovarian development?

A

Two X chromosomes

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9
Q

What two hormones are not secreted in development of females?

A

testosterone or mullerian inhibiting factor

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10
Q

The absence of this factor in developing female fetus leads to differentiation of mullerian ducts to ovaries, uterus and other reproductive tract organs.

A

mullerian inhibiting factor

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11
Q

The absence of this hormone in the developing female fetus leads to undifferentiated external female genitalia, and the degeneration of wolffian ducts

A

testosterone

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12
Q

the testes of a developing male embryo secrete what two substances?

A

testosterone and mullerian inhibiting factor

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13
Q

The secretion of mullerian inhibiting factor in the developing male fetus leads to what

A

degeneration of mullerian ducts

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14
Q

Some of the testosterone secreted by the testes of developing male fetuses is converted to…

A

dihydrotestosterone

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15
Q

Converted dihydrotestosterone in the developing male fetus has what effect?

A

promotes male external genitalia

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16
Q

Testosterone secreted from the testes in the developing male embryo can be converted to dihydrotestosterone, or remain as T. What effect does T have on the fetus?

A

transforms wolffian ducts to male reproductive tract

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17
Q

During the first 5 weeks, gonads are…

A

indifferent/bipotential

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18
Q

In weeks 6-7 in genetic males, what happens that influences gonadal development?

A

SRY produces TDF

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19
Q

In the absence of SRY in weeks 6-7, by week 9 what happens?

A

female gonadal development begins

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20
Q

What three cell types do the developing testes have?

A
  1. germ cells (spermatogonia
  2. sertoli cells
  3. leydig cells
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21
Q

sertoli cells in the developing male testes produce…

A

anti mullerian hormone

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22
Q

leydig cells in the developing male testes produce…

A

T

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23
Q

Ovaries in developing females have what three cell types?

A
  1. germ cells (oogonia
  2. granulosa cells
  3. theca cells
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24
Q

Granulosa cells in developing ovaries produce…

A

estradiol

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25
Q

Theca cells in developing ovaries produce…

A

androgens and progesterone

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26
Q

______ ducts differentiate into male reproductive tract while _____ ducts degenerate

A

Wolffian ducts = male

mullerian ducts degenerate = male

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27
Q

______ ducts differentiate into female reproductive tract while ______ ducts degenerate.

A

mullerian ducts = female

wolffian ducts degenerate

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28
Q

The epididymis, vas deferens, seminal vesicles and ejaculatory ducts come from what structure?

A

wolffian ducts

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29
Q

Differentiation of the external genetalia in males occurs around weeks 9-10 as what hormone is secreted?

A

DHT

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30
Q

In the absence of DHT, what happens to the genitalia?

A

female-like external genetalia

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31
Q

Are hormones needed to cause the development of female gonads?

A

no

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32
Q

What is biochemically required in order to develop female gonads?

A

to X chromosomes

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33
Q

Fallopian tubes, uterus and upper 1/3 of the vagina develop when this structure remains intact…

A

mullerian ducts

34
Q

The lower 2/3 of the vagina, clitoris and labia (do/don’t) require hormones for development

A

don’t

35
Q

What effects the growth to normal size of female external genitalia?

A

estradiol

36
Q

this disorder has the following characteristics:

  • XY chromosomes
  • Presence of testes with secretion of AMH and testosterone
  • Regression of wolffian and mullerian ducts
  • female external genitalia, but blind ended vagina
A

complete androgen insensitivity syndrome

37
Q

How is complete androgen insensitivity syndrome diagnosed in infancy and at puberty

A

infancy: bilaterall inguinal mass
puberty: amenorrhea

38
Q

XY lacking SRY would cause what?

A

no gonads, develop as female

39
Q

XX with SRY translocation would have what effect on development?

A

testes, develop as male

40
Q

XY with defective AMH production/action would have what 3 effects?

A

testes

both male and female internal genitalia

male external genitalia

41
Q

XY with absence of testosterone production/action would have what 3 effects?

A

Testes

no male/female internal genitalia

female-like external genitalia

42
Q

5-alpha reductase deficiency would have what 2 effects?

A

Testes

male internal genitalia with female external genitalia

(aka male pseudohemaphrotidism)

43
Q

What age range is normal for puberty in boys?

A

9-14

44
Q

what age range is normal for puberty in girls?

A

8-13

45
Q

Puberty is characterized by the following, which occurs when what anatomical structure matures?

-appearance of secondary sex characteristics

acceleration of growth

capacity for fertilization

A

hypothalamic-pituitary-gonadal axis maturation

46
Q

Around 13.5-13.7 yo, what 2 important processes occur in boys?

A

sperm production

ejaculatory capability

47
Q

This marks the start of puberty, when testicles increase in size, along with development of pubic hair and penile enlargement…

A

gonadarche

48
Q

Adult testicular volume and penile size is achieved by what age in boys?

A

16

49
Q

Final height of boys occurs at what age?

A

18

50
Q

In girls, what marks the start of puberty at age 10.9?

A

thelarche (breast development)

51
Q

What follows thelarche at age 11.2 in females?

A

pubarche

52
Q

Menarch usually occurs by what age?

A

13

53
Q

are the first few menstrual cycles ovulatory? why?

A

non-ovulatory

no estrogen positive feedback

54
Q

males have ___x higher skeletal and muscle mass

A

1.5

55
Q

females have ____x fat as males

A

2x

56
Q

What two hormones are important for growth spurt?

A

GH and IGF-1

57
Q

_____ have their growth spurt early in puberty

A

females

58
Q

_____ have their growth spurt towards the end of puberty

A

males

59
Q

_____ promotes epiphyseal fusion towards the end of puberty in both males and females

A

estrogen

60
Q

high levels of this hormone may lead to shorter stature

A

estrogen

61
Q

What accounts for the difference in stature between males and females?

A

males take longer to reach puberty

62
Q

This hypothesis of pubertal timing posits that a reduction in intrinsic GnRH suppression is responsible for onset of puberty

A

hypothalamic maturation hypothesis

63
Q

This hypothesis of pubertal timing posits that decreased sensitivity to estrogen/testosterone negative feedback is responsible for the onset of puberty…

A

Gonadostat hypothesis

64
Q

The following are responsible for…

  • reactivation of gonadotropin synthesis/secretion
  • increased pulsatile GnRH release
A

pubertal timing

65
Q

Increased ratio of _____ release during sleep in both sexes correlates with the onset of early puberty

A

increased LH/FSH ratio

66
Q

The major determinant of pubertal timing is..

A

genetic

67
Q

is weight, or body fat a bigger determinant of pubertal timing?

A

body fat (16%-23.5)

68
Q

What theory of the signal for puberty?

  • puberty gated by energy resources of body
  • accellerated in obesity
  • metabolic signal from leptin secreted from adipose tissue controls onset of maturation
A

Leptin theory

69
Q

Mutant mice unable to produce this hormone fail to undergo normal puberty…

A

leptin

70
Q

What theory of the signal for puberty?

-may be initiated by a reduction in a hormone secreted by the pineal gland which inhibits GnRH release

A

Melatonin theorgy

71
Q

Removal of the _______ precipitates puberty…

A

pineal gland

72
Q

This pubertal disorder…

  • increased gonadotropins (LH, FSH)
  • 5x more common in girls
  • puberty begins before 8 in girls, or 9 in boys
  • MC idiopathic in nature or caused by CNS tumor
A

Gonadotropin-dependent precocious puberty

73
Q

This pubertal disorder…

  • normal gonadotropins, increased gonadal hormones
  • testicular disorders caused by androgen secreting tumors
  • ovarian disorders from estrogen secreting tumors
  • puberty begins before 8 in girls, before 9 in boys
A

gonadotropin-independent precocious puberty

74
Q

What is the tx for gonadotropin-dependent precocious puberty?

A

long acting GnRH agonists

75
Q

This mechanism describes what drug class?

  • initial release of FSH and LH, followed by desensitization of receptors
  • leads to reduced gonadotropins, sex steroids and biologic effects
A

long-acting GnRH agonists

76
Q

The following presentation describes…

  • 6 yo vaginal bleeding
  • tanner stage 4
  • estrogen producing ovarian cyst
A

Gonadotropin independent precocious puberty

77
Q

What is the tx for gonadotropin independent precocious puberty?

A

surgical removal of tumor

78
Q

What disorder of delayed puberty?

  • gonadotropin deficiency leading to low gonadal hormones
  • deficiency in pulsatile release of gonadotropins
  • Kallman’s syndrome
A

Hypogonadotropic hypogonadism

79
Q

What disorder of delayed puberty?

  • Primary gonadal failure
  • low gonadal hormones result in high gonadotropins due to lack of negative feedback
  • gonadal streak
  • turner’s syndrome and klinefelter’s syndrome
A

hypergonadotropic hypogonadism

80
Q

Which disorder?

  • failure of fetal migration of GnRH neurons to hypothalamus
  • associated with anosmia, lack of puberty, short stature

Tx with supplemental sex-steroids

A

Kallman’s syndrome

81
Q

Which disorder?

  • female tract forms
  • no functional gonads

associated with short stature, delayed or absent puberty

tx with GH then sex steroids

A

Turner’s syndrome (XO)

82
Q

Which disorder?

  • MC form of primary testicular failure due to no negative feedback
  • XXY genotype
  • feminization occurs
  • germ cell tumors, breast cancer, osteoporosis can occur

Tx with GH then supp. sex steroids

A

Klinefelter’s syndrome