Puberty Flashcards

1
Q

What are the 3 types of “sex”

A

Genetic- XX or XY

Gonadal- do you have testes or ovaries

Phenotypic- do you have a penis or labia

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2
Q

What part of the Y chromosome turns you into a boy

A

The SRY (Sex-determing Region of the Y). It makes TDF (Testis Determining Factor) which promotes testis differentiation

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3
Q

How many X chromosomes are necessary for ovarian development/

A

Two

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4
Q

What causes the undifferentiated gonads to developinto ovaries

A

The absence of TDF

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5
Q

What are the two ducts that undifferentiated reproductive organs have?

A

Wolffian ducts- become male reproductive tract (epididymis, vas deferens, etc)

Müllerian ducts- become female reproductive tract (Fallopian tubes, uterus)

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6
Q

What causes the Müllerian ducts to degenerate in a baby boy

A

Anti-mullerian hormone***

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7
Q

What causes the wolffian ducts to degenerate in a baby girl?

A

Absence of testosterone**

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8
Q

What enzyme converts Testosterone to Dihydrotestosterone (DHT)?

A

5-α-reductase**

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9
Q

What does DHT do for a developing baby boy

A

Promotes the development of EXTERNAL genitalia (penis, scrotum)

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10
Q

What causes the Müllerian ducts to develop into Fallopian tubes and a uterus in a baby girl?

A

The absence of anti-mullerian hormone.

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11
Q

What causes the development of female EXTERNAL genitalia (labia, clitoris)

A

Absence of testosterone

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12
Q

What are the 3 cell types in testes

A

Germ cells (spermatogonia)

Sertoli cells (produce anti-mullerian hormone)

Leydig cells (produce testosterone)

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13
Q

What are the 3 cell types in ovaries?

A

Germ cells (oogonia)

Granulosa cells (produce estradiol)

Theca cells (produce androgens and progesterone)

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14
Q

Can you look at a 5 week old fetus and tell what gender it is?

A

No, testes develop in week 6 and ovaries develop in week 9

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15
Q

What hormones are needed to cause the development of the female gonads, the reproductive tract, and the eternal genitalia

A

NONE AT ALL!!!***

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16
Q

What part of the vagina comes from the Müllerian ducts vs the “external” part

A

Upper 1/3 comes form Müllerian ducts

Lower 2/3 is the “external” genitalia

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17
Q

What will happen if a developing baby girl is exposed to high levels of androgens in utero?

A

External genitalia will differentiate into a male-like phenotype!***

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18
Q

What is Congenital Adrenal Hyperplasia aka 21-hydroxylase deficiency aka Virilixed X syndrome?

A

No 21-hydroxylase enzyme

No aldosterone or cortisol

Excessive ACTH results in the adrenal cortex eating huge and making excessive androgens

Individuals are XX with ovaries and female internal genitalia

Female external genitalia develop but VIRILIZE= clitoris grows to look like a penis, and labial folds appear to be an empty scrotum

Life threatening adrenal insufficiency

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19
Q

What is the most common cause of genital ambiguity

A

Congenital adrenal hyperplasia/21-hydroxylase deficiency

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20
Q

What do people with congenital adrenal hyperplasia/21-hydroxylase deficiency look like

A

XX with ovaries, uterus, Fallopian tubes and top 1/3 of vagina

External genitalia is female, but viralized. Big clitoris, labia looks like scrotum

Acne

Hirsutism 🧔🏻

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21
Q

What happens if an XY person lacked the SRY gene?

A

No gonads

Develops as female

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22
Q

What happens if an XX person gets a translocation of the SRY gene

A

Testes

Develops as male

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23
Q

What happens if an XY person has defective AMH production or action

A

Testes

Male and female internal genitalia (wolffian and Müllerian ducts develop)

Male external genitalia

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24
Q

What happens if someone is XY with no testosterone production/action?

A

Testes

No internal genitalia

Female external genitalia

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25
Q

What would happen in 5α-reductase deficiency?

A

Testes

Male internal genitalia

Female external genitalia

(Male pseudohermaphroditism)

testosterone not converted into DHT
*******she really talked about this one a lot

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26
Q
What laboratory findings would you see with Complete Androgen Insensitivity Syndrome?
Testosterone
DHT
FSH and Inhibin
LH
AMH
XX or XY?
Ultrasound:
Genetic skin fibroblast biopsy
A

Testosterone: high

DHT: normal

FSH and Inhibin: normal

LH: high

AMH: high (must mean there are testes..)

XX or XY? XY

Ultrasound: no ovaries/uterus. Male testes

Genetic skin fibroblast biopsy: absence of binding affinity of testosterone to androgen receptors

External appearance is normal female

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27
Q

What causes peopel with Complete Androgen Insensitivy syndrome to have NO internal genitalia?

A

They have testes that secrete AMH and testosterone (but the testosterone is not doing anything to support the Wolffian ducts)

Both wolffian and Müllerian ducts regress

Female external genitalia develop, but have a blind ended vagina, and testes

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28
Q

Is complete Androgen insensitivity syndrome comepletely black and white?

A

No there are variants. May be mild, partial or complete

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29
Q

What is the treatment for Complete Androgen Insensitivity Syndrome?

A

Gonadectomy

Estrogen replacement therapy

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30
Q

In older ages, FSH and LH are both increased at higher levels than any other time in life. Which one is always higher?

A

FSH is always higher than LH

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31
Q

during reproductive years, which hormone is higher: LH or FSH

A

LH is higher

32
Q

During childhood, which is higher: LH or FSH

A

FSH is higher than LH

The only time LH is higher is during reproductive years

33
Q

When are the biggest peaks of GnRH?

A

Embryonic development

Infancy

Reproductive years in women (monthly cycles)

34
Q

What marks the start of puberty in boys?

A

Increasein testicular size (gonadarche) followed by development of pubic hair and penile enlargement

35
Q

What do boys develop sperm production and ejaculatory capability?

A

13.5-13.7 years old ***

HAD A RED BOX AROUND IT

36
Q

What marks the start of puberty in girls?

A

Thelarche (breast development) Mean age: 10.9 years

37
Q

When do girls start mensrtuating?

A

Usually by 13th birthday

38
Q

Can a girl get pregnant during her first few menstrual cycles?

A

No!!!**

There is no ovulation for the first few cycles because there is no positive feedback by estrogen**

39
Q

What is the order of things developing in a girl

A

Thelarche

Pubarche (pubic hair) 11.2 yrs

Menarche

Adipose in hips

Hips enlarge, pelvic inlet widens

40
Q

When do girls have their growth spurt

A

Begins in early puberty and is complete by menarche**

41
Q

When do boys have their growth spurt?

A

Begins near the end of puberty, almost 2 years later than in girls*****

42
Q

What hormone fuses epiphyses in males AND females

A

Estrogen

43
Q

What happens to your height if you have EXCESSIVE estrogen during puberty

A

Shorter stature. (Estrogen fuses epiphyses toward the end of puberty in males and females)

44
Q

How does puberty affect your dental health

A

May be the first appearance of periodontal disease due to mouth flora changing

45
Q

Why are boys taller than girls

A

Longer time to reach puberty in boys accounts for most of the difference in stature

46
Q

What causes puberty to begin?

A

Increase in pulsatile GnRH release that occurs during sleep

Causes MORE LH THAN FSH
*******
LH is stimulated by HIGH pulse frequency

47
Q

What is the major determinant of pubertal timing?

A

Genetics

48
Q

Which is more important than weight in determining timing of puberty?

A

Body fat percentage ***

Fat kids go into puberty earlier

49
Q

What is the leptin theory for signaling puberty?

A

Puberty is physically gated by the energy resources of the body.

Leptin (which comes from adipose tissue) may control onset of puberty.

Delayed puberty in female ballet dancers

Accelerated puberty in obese females

50
Q

What happens when mice can not produce leptin?

A

They never undergo puberty.

As soon as they are given leptin, all aspects of the reproductive system are stimulated and fertility ensues

51
Q

What is the melatonin theory in regard to signaling puberty to start?

A

Melatonin inhibits GnRH release***

Puberty may be initiated by a reduction in melatonin secretion

LH and FSH spike ONLY at night time during puberty, which makes us think melatonin may be related

52
Q

LH is higher than FSH ONLY during ______________

A

Reproductive years

53
Q

What is precocious puberty?

A

Development of secondary sexual characteristics before age 8 in girls or 9 in boys

54
Q

What are the 2 types of precocious puberty?

A

Gonadotropin-dependent

Gonadotropin-independent

55
Q

What is gonadotropin-dependent precocious puberty?

A

🧠Increased gonadotropins (LH, FSH) cause an early onset of puberty

Can be idiopathic or caused by brain tumor

56
Q

What is gonadotropin-independent precocious puberty?

A

Gonadotropons are normal, but gonadal hormones are increased.

Due to androgen secreting tumors in testes or estrogen secreting tumors in ovaries 🥚🥚

57
Q

How do you treat gonadotropin-dependent precocious puberty?

A

Long-acting GnRH agonists****

Occupies the receptors, causes an initial release of FSH and LH, followed by down-regulation and desensitization

58
Q

Do boys are girls get gonadotropin-dependent precocious puberty more often

A

Girls 5x more

59
Q

What is the treatment for gonadotropin-independent precocious puberty?

A

Surgical tumor removal

60
Q

What is delayed puberty?

A

Lack of physical maturation 2 standard deviations beyond the mean onset

61
Q

What are the 2 types of delayed puberty

A

Hypogonadotropic hypogonadism

Hypergonadotropic hypogonadism

62
Q

What is hypogonadotropic hypogonadism?

A

Gonadotropin deficiency***🧠

Deficiency of pulsatile release of gonadotropins

A cause of Kallman’s syndrome
*******

63
Q

What is hypergonadotropic hypogonadism?

A

Low gonadal hormones result in high gonadotropins due to lack of negative feedback

Streaked gonads

Cause Turners and Klinefelters
***

64
Q

What is Kallman’s syndrome?

A

No GnRH neurons in hypothalamus= no puberty

Associated with ansomia due to a genesis of the olfactory bulb👃

65
Q

No puberty

Can’t smell anything

Short stature

A

Kallman’s syndrome

66
Q

How do you treat Kallman’s syndrome?

A

Supplemental sex-steroid (estrogen or testosterone) and then GnRH later for reproductive capability

67
Q

What is Turner’s syndrome

A

Person is XO

Female genital tract and external genitals but NO FUNCTIONAL GONADS
=no negative feedback so LH and FSH will be HIGH

Amenorrhea

68
Q

How do you treat Turners Syndrome

A

Give GH first for height, then supplemental sex steroids for secondary sex characteristics

69
Q

Female genitalia and genital tract

No functional gonads

Short stature

No/late puberty

Amenorrhea

A

Turner’s Syndrome XO

70
Q

What is Klinefelter’s syndrome?

A

47, XXY male

Primary testicular failure=no negative feedback

Feminization occurs 💄

May have germ cell tumors, breast cancer, and osteoporosis

71
Q

What is the MOST common form of primary testicular failure?

A

Klinefelter’s Syndrome

***

72
Q

How do you treat Klinefelters Syndrome?

A

GH first, then supplemental sex steroids

73
Q

How do you solve the test problems of pubertal disorders?

A
  1. Is puberty delayed/precocious? Will tell you if gonadal hormones are high or low
  2. Are there normal levels of gonadotropins?
74
Q

Gonadotropin-dependent precocious puberty

LH/FSH levels:

Gonadal hormone levels:

A

LH/FSH: increased

Gonadal hormones: increased

75
Q

Gonadotropin-independent precocious puberty

LH/FSH levels:

Gonadal hormone levels:

A

LH/FSH: normal

Gonadal: high

76
Q

Hypogonadotropic Hypogonadism

LH/FSH levels:

Gonadal hormones:

A

LH/FSH: low

Gonadal hormones: low

(Delayed puberty)

77
Q

Hypergonadotropic hypogonadism

LH/FSH levels:

Gonadal hormones:

A

LH/FS: high

Gonadal hormones: low

(Delayed puberty)