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
What would happen in 5α-reductase deficiency?
Testes Male internal genitalia Female external genitalia (Male pseudohermaphroditism) testosterone not converted into DHT ***************she really talked about this one a lot
26
``` 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 ```
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
27
What causes peopel with Complete Androgen Insensitivy syndrome to have NO internal genitalia?
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
28
Is complete Androgen insensitivity syndrome comepletely black and white?
No there are variants. May be mild, partial or complete
29
What is the treatment for Complete Androgen Insensitivity Syndrome?
Gonadectomy Estrogen replacement therapy
30
In older ages, FSH and LH are both increased at higher levels than any other time in life. Which one is always higher?
FSH is always higher than LH
31
during reproductive years, which hormone is higher: LH or FSH
LH is higher
32
During childhood, which is higher: LH or FSH
FSH is higher than LH | The only time LH is higher is during reproductive years
33
When are the biggest peaks of GnRH?
Embryonic development Infancy Reproductive years in women (monthly cycles)
34
What marks the start of puberty in boys?
Increasein testicular size (gonadarche) followed by development of pubic hair and penile enlargement
35
What do boys develop sperm production and ejaculatory capability?
13.5-13.7 years old ******* HAD A RED BOX AROUND IT
36
What marks the start of puberty in girls?
Thelarche (breast development) Mean age: 10.9 years
37
When do girls start mensrtuating?
Usually by 13th birthday
38
Can a girl get pregnant during her first few menstrual cycles?
No!!!**** There is no ovulation for the first few cycles because there is no positive feedback by estrogen**********
39
What is the order of things developing in a girl
Thelarche Pubarche (pubic hair) 11.2 yrs Menarche Adipose in hips Hips enlarge, pelvic inlet widens
40
When do girls have their growth spurt
Begins in early puberty and is complete by menarche****
41
When do boys have their growth spurt?
Begins near the end of puberty, almost 2 years later than in girls*****
42
What hormone fuses epiphyses in males AND females
Estrogen
43
What happens to your height if you have EXCESSIVE estrogen during puberty
Shorter stature. (Estrogen fuses epiphyses toward the end of puberty in males and females)
44
How does puberty affect your dental health
May be the first appearance of periodontal disease due to mouth flora changing
45
Why are boys taller than girls
Longer time to reach puberty in boys accounts for most of the difference in stature
46
What causes puberty to begin?
Increase in pulsatile GnRH release that occurs during sleep | Causes MORE LH THAN FSH *************** LH is stimulated by HIGH pulse frequency
47
What is the major determinant of pubertal timing?
Genetics
48
Which is more important than weight in determining timing of puberty?
Body fat percentage ******* Fat kids go into puberty earlier
49
What is the leptin theory for signaling puberty?
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
What happens when mice can not produce leptin?
They never undergo puberty. As soon as they are given leptin, all aspects of the reproductive system are stimulated and fertility ensues
51
What is the melatonin theory in regard to signaling puberty to start?
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
LH is higher than FSH ONLY during ______________
Reproductive years
53
What is precocious puberty?
Development of secondary sexual characteristics before age 8 in girls or 9 in boys
54
What are the 2 types of precocious puberty?
Gonadotropin-dependent Gonadotropin-independent
55
What is gonadotropin-dependent precocious puberty?
🧠Increased gonadotropins (LH, FSH) cause an early onset of puberty Can be idiopathic or caused by brain tumor
56
What is gonadotropin-independent precocious puberty?
Gonadotropons are normal, but gonadal hormones are increased. Due to androgen secreting tumors in testes or estrogen secreting tumors in ovaries 🥚🥚
57
How do you treat gonadotropin-dependent precocious puberty?
Long-acting GnRH agonists****** Occupies the receptors, causes an initial release of FSH and LH, followed by down-regulation and desensitization
58
Do boys are girls get gonadotropin-dependent precocious puberty more often
Girls 5x more
59
What is the treatment for gonadotropin-independent precocious puberty?
Surgical tumor removal
60
What is delayed puberty?
Lack of physical maturation 2 standard deviations beyond the mean onset
61
What are the 2 types of delayed puberty
Hypogonadotropic hypogonadism Hypergonadotropic hypogonadism
62
What is hypogonadotropic hypogonadism?
Gonadotropin deficiency***🧠 Deficiency of pulsatile release of gonadotropins A cause of Kallman’s syndrome *********
63
What is hypergonadotropic hypogonadism?
Low gonadal hormones result in high gonadotropins due to lack of negative feedback Streaked gonads Cause Turners and Klinefelters *******
64
What is Kallman’s syndrome?
No GnRH neurons in hypothalamus= no puberty Associated with ansomia due to a genesis of the olfactory bulb👃 ***********
65
No puberty Can’t smell anything Short stature
Kallman’s syndrome
66
How do you treat Kallman’s syndrome?
Supplemental sex-steroid (estrogen or testosterone) and then GnRH later for reproductive capability
67
What is Turner’s syndrome
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
How do you treat Turners Syndrome
Give GH first for height, then supplemental sex steroids for secondary sex characteristics
69
Female genitalia and genital tract No functional gonads Short stature No/late puberty Amenorrhea
Turner’s Syndrome XO
70
What is Klinefelter’s syndrome?
47, XXY male Primary testicular failure=no negative feedback Feminization occurs 💄 May have germ cell tumors, breast cancer, and osteoporosis *****
71
What is the MOST common form of primary testicular failure?
Klinefelter’s Syndrome | ***********
72
How do you treat Klinefelters Syndrome?
GH first, then supplemental sex steroids
73
How do you solve the test problems of pubertal disorders?
1. Is puberty delayed/precocious? Will tell you if gonadal hormones are high or low 2. Are there normal levels of gonadotropins?
74
Gonadotropin-dependent precocious puberty LH/FSH levels: Gonadal hormone levels:
LH/FSH: increased Gonadal hormones: increased
75
Gonadotropin-independent precocious puberty LH/FSH levels: Gonadal hormone levels:
LH/FSH: normal Gonadal: high
76
Hypogonadotropic Hypogonadism LH/FSH levels: Gonadal hormones:
LH/FSH: low Gonadal hormones: low (Delayed puberty)
77
Hypergonadotropic hypogonadism LH/FSH levels: Gonadal hormones:
LH/FS: high Gonadal hormones: low (Delayed puberty)