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OBGYN Clerkship > Puberty, Androgenism, and Hirsuitism > Flashcards

Puberty, Androgenism, and Hirsuitism Flashcards

1
Q

Adrenarche normally begns at age __ in girls

Menarche normally begns at age __ in girls

A

Adrenarche normally begns at age 6-8 in girls

Menarche normally begns at age 12-14 in girls

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2
Q

Sequence of events in puberty in normal physiology

A
  1. Growth acceleration
  2. Thelarche (breast development, stimulated by estradiols)
  3. Pubarche (beginning of pubic hair growth, stimulated by androgens)
  4. Maximum growth rate
  5. Menarche
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3
Q

If a girl with abnormal puberty fails to undergo pubarche, the problem is likely ___.

If a girl with abnormal puberty fails to undergo thelarche, the problem is likely ___.

A

If a girl with abnormal puberty fails to undergo pubarche, the problem is likely failure of adrenal androgen production.

If a girl with abnormal puberty fails to undergo thelarche, the problem is likely failure of estrogen production.

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4
Q

Mild to moderate obesity results in __ puberty

A

Mild to moderate obesity results in early puberty

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5
Q

Puberty and depression

A

Pre-pubertal rates of depression are equal among boys and girls, however among age-matched boys and girls undergoing puberty, girls are disproportionately affected by pubertal depression.

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6
Q

Precocious puberty “line”

A
  • Weirdly, it is different by ethnicity
    • Prior to age 6 for girls of African American heritage
    • Prior to age 7 for girls of European heritage
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7
Q

Gn-RH dependent precocious puberty etiologies

A
  • Mostly idiopathic
  • Infection/inflammation of the CNS
  • Rarely a tumor of the hypothalamic-pituitray stalk
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8
Q

Characteristic features of McCune Albright syndrome

A
  1. Café-au-lait spots with ragged edges
  2. Polyostotic fibrous dysplasia (bones replaced by fibrous tissue)
  3. Endocrinopathies (precocious puberty, cushing syndrome, acromegaly, hyperthyroidism)
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9
Q

The 3 P’s of McCune-Albright syndrome

A
  • Polyostotic fibrous dysplasia
  • Pigmentation (café-au-lait spots)
  • Precocious puberty.
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10
Q

GnRH-independent precocious puberty

A
  • McCune Albright Syndrome:
    • Ovaries produce estrogen without FSH signaling
    • Multiple bone factures
    • Cafe-au-lait spots
  • Estrogen-secreting tumor (granulosa cell)
  • Adrenal hormone-secreting tumor
  • Congenital adrenal hyperplasia
  • Iatrogenic (OCP)
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11
Q

Rapid progression in precocious puberty is more likely to be. . .

A

. . . GnRH independent

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12
Q

Laboratory test to differentiate GnRH-dependent and -independent precocious puberty

A

Baseline LH is the standard. If high, GnRH dependence is already confirmed. If low, GnRH stimulation (non-pulsatile!!!) can tell you if LH is responding appropriately (inhibited) or not (elevated).

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13
Q

Once you determine that precocious puberty is GnRH dependent, the next step is. . .

A

. . . brain imaging

To visualize the pituitary

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14
Q

Delayed puberty categories

A
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15
Q

Most common cause of hypergonadotropic hypogonadism

A

Turner’s syndrome

FSH will be high. If missed at birth, these patients usually present with primary amenorrhea.

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16
Q

Treating hypergonadotropic hypogonadism

A

Usually involves supplementing the estrogen. So, basically, OCP.

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17
Q

Causes of hypogonadotropic hypogonadism

A
  • Constitutional delay (most common, often familial)
  • Kallman’s syndrome (hypogonadotropic hypogonadism plus poor olfactory tracts/no sense of smell)
  • Anorexia (Leptin-mediated)
  • Exercise (Athlete’s triad)
  • Stress
  • Craniopharyngioma
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18
Q

17-alpha-hydroxylase deficiency

A

Rare form of CAH that causes BOTH androgeneic effects AND mineralocorticoid effects

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19
Q

Specific definition of hirsuitism

A
  • Excess terminal hair in a male pattern of distribution
    • Terminal hair is dark and coarse. Develops first during puberty.
    • Vellus hair is soft, downy, and fine. Present from birth.
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20
Q

Specific definition of virilization

A
  • “Masculinization” of a woman
    • Enlargement of clitoris
    • Temporal balding
    • Deepening of voice
    • Involution of breasts
    • Remodeling of limb-shoulder girdle
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21
Q

Four important forms of CAH

A
  • Common CAH genotypes:
    • 21-hydroxylase deficiency
    • 11-beta-hydroxylase deficiency
  • CAH plus salt wasting
    • 3-beta-hydroxysteroid-dehydrogenase deficiency
  • CAH plus hyperaldosteronism:
    • 17-alpha-hydroxylase deficiency
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22
Q

Ddx for someone who develops secondary amenorrhea with hirsuitism and virilization at age 20

A
  • PCOS
  • Cushing’s syndrome
  • Hyperprolactinemia
  • Acromegaly
  • Hypothyroidism
  • Atypical CAH
  • Sertoli-Leydig tumor
    • It is really important not to forget the last two because they are less common but potentially dangerous – moreso than PCOS
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23
Q

Ferriman-Gallway score

A

Score quantifying the presence of hirsuitism in 9 different locations on a female to assess for the presence of hirsuitism in that individual.

Note that ethnicity is relevant here, since different populations have different patterns of normal hair distribution (for example, women of Asian descent express no androgen receptors on their face, so they will never have hirsuitism there even when they have sky high androgens)

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24
Q

Eflornithine hydrochloride

A
  • Facial cream approved for treatment of facial hirsuitism
  • Inhibits the anagen phase of hair production
  • Irreversible inhibitor of ornithine decarboxylase
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25
Q

Anti-hirsuitism medications

A
  • Eflornithine hydrochloride (topical)
  • OCP
  • Spironolactone
  • Finasteride
26
Q

What do elevated testosterone, ACTH, cortisol, and DHEAS tell you about the source of the androgen hormones in a female patient with hirsuitism?

A
  • Testosterone: Source is the ovary
  • ACTH, cortisol, DHEAS: Source is the adrenal gland
27
Q

Ovarian androgen synthesis diagram

A
28
Q

___ express LH-R in the ovaries, which activates PKA to turn on ___.

___ express FSH-R in the ovaries, which activates PKA to turn on ___.

A

Theca cells express LH-R in the ovaries, which activates PKA to turn on androstenedione synthesis.

Granulosa cells express FSH-R in the ovaries, which activates PKA to turn on aromatase (conversion of androstenedione to estrone and estriol).

29
Q

Adrenal gland diagram

A
30
Q

OCP progestins that have less androgeneic properties

A
  • Desogestrel
  • Gestodene
  • Norgestimate
31
Q

Effects of estrogen on 5-alpha reductase

A

Decreases the activity of 5-alpha reductase, preventing the conversion of testosterone to dihydrotestosterone

This is why estrogen-containing OCP is so effective for treating male-pattern balding in women

32
Q

Medroxyprogesterone

A

Decreases GnRH, resulting in decreased testosterone production

33
Q

If hirsuitism is caused by adrenal hyperandrogenism, then ___ is the drug of choice.

A

If hirsuitism is caused by adrenal hyperandrogenism, then a glucocorticoid is the drug of choice.

34
Q

Ketoconazole’s interaction with the androgen system

A

Inhibits steroidogenic cytochrome enzymes, resulting in decreased free testosterone and androstenedione

35
Q

5-alpha reductase deficiency

A
  • Classical presentation is a pre-pubertal female that becomes male at puberty (development of vulvar enlargement, penoid clitoris)
    • Happens this way because the end effects of testosterone are mediated mostly by DHT. Prior to puberty, testosterone levels are too low to overcome the low enzyme activity. But at puberty, testosterone surge produces significant quantities of DHT, which then produce male secondary sex characteristics.
  • Three sexual differentiations:
    • Genetic: Male
    • Gonadal: Male
    • Genital: Female
36
Q

Obstructive azoospermia

A
  • Will be associated with low fructose (added from seminal vesicles)
  • Most commonly congenital vas deferens absence due to cystic fibrosis
37
Q

Non-classical CAH

A
  • Presents very similarly to PCOS
  • Pubertal or post-pubertal onset of hirsuitism and virilization
  • Screen w/ DHEAS and 17 hydroxyprogesterone. Diagnose w/ cosyntropin stimulation w/ 17-OH PG as readout.
  • Three sexual differentiations:
    • Genetic: Female
    • Gonadal: Female
    • Genital: Female (unlike classical CAH, which would be male)
38
Q

Mosaic Turner’s

A

Mixed phenotype.

Will have a late onset, be associated with normal stature, and may cause secondary amenorrhea.

Karyotype will be mixed 45X/46XX/47XXX.

39
Q

Timing of gonadal vs genital differentiation of sex during embryonic development

A

Gonadal: 6 weeks EGA, SRY-dependent

Genital: 8-12 weeks EGA, testosterone and AMH-dependent

40
Q

Sertoli cells vs Leydig cells

A

Sertoli: Produce AMH, stimulate mullerian agenesis

Leydig: Produce testosterone, stimulate wolffian differentiation

41
Q

XY Gonadal agenesis

A
  • Genetically XY, but SRY is nonfunctional or otherwise insufficient
  • Testes do not develop properly or at all
    • If any gonadal tissue develops, it must be removed. High risk of progression to malignancy
  • Three sexual differentiations:
    • Genetic: Male
    • Gonadal: None/Malformed Male
    • Genital: Female
42
Q

Androgen insensitivity syndrome

A
  • XY w/ androgen receptor deficiency
  • No wolffian or mullerian structures, otherwise phenotypically female
  • Gonadectomy recommended after puberty
  • Three sexual differentiations:
    • Genetic: Male
    • Gonadal: Male
    • Genital: Female
43
Q

Mullerian agenesis

A
  • 46XX karyotype
  • Etiology unknown
  • Mullerian system spontaneously regresses, but ovaries are still functional.
  • Three sexual differentiations:
    • Genetic: Female
    • Gonadal: Female
    • Genital: None/Malformed Female
44
Q

Kallman syndrome

A
  • May be 46XX or 46XY
  • No GnRH neurons or sense of smell (anosmia)
  • Three sexual differentiations:
    • Variable, but there will be no spontaneous secondary sex characteristics apart from adrenarche and no spontaneous menarche in females
45
Q

Androgen insensitivity syndrome produces a phenotypically female invidual with a karyotype of ___ and ___ testosterone.

Mullerian agenesis produces a phenotypically female invidual with a karyotype of ___ and ___ testosterone.

A

Androgen insensitivity syndrome produces a phenotypically female invidual with a karyotype of XY and High testosterone.

Mullerian agenesis produces a phenotypically female invidual with a karyotype of XX and Low testosterone.

46
Q

Rough picture for Tanner stages

A

Stage 1: Prepubertal

2: Few pubic hairs (just a couple)
3: Minimal pubic hairs (still countable)
4: Sparse pubic hair (no longer countable)
5: Adult pubic hair

47
Q

Order of events in female puberty

A
  1. Thelarche
  2. Growth spurt / peak growth velocity (1 year later)
  3. Adrenarche
  4. Menarche
48
Q

McCune Albright syndrome

A
  • Three P’s: Precocious puberty, pigmented skin lesions (cafe-au-lait), and polyostic fibrous dysplasia
  • Constant production of estrogen by ovaries due to cellular signaling defect. Form of peripheral precocious puberty.
  • Treat w/ letrozole (aromatase inhibitor)
49
Q

First test you should order in any patient with precocious puberty

A

Bone age!!!!

This confirms the presence of true precocious puberty. If you can only get one test, get this one.

If we don’t intervene, epiphyseal plates will close and they will never reach adult height.

50
Q

Classical CAH

A
  • Autosomal recessive
  • Genes:
    • 21-hydroxylase (90% of cases)
    • 11 beta hydroxylase (associated with hypertension, mineralocorticoid effects)
    • 3 beta hydroxysteroid dehydrogenase
  • In-utero virilization of fetus at 6-7 weeks
  • If suspected in a pregnancy, give dexamethasone immediately (if prior to or at 6-7 wks). You can stop if the sex is confirmed as male. This will prevent abnormal male genital development in a genetically female fetus.
51
Q

“Flow” of adrenal steroid pathway

A
52
Q

The very first manifestation of puberty in girls

A

Growth acceleration

53
Q

17-hydroxyprogesterone in different forms of CAH

A
  • In 21-hydroxylase deficiency and 11-beta-hydroxylase deficiency, the most classical forms of CAH, 17-OHP is markedly elevated.
  • In 3-beta-hydroxysteroid-reductase deficiency, 17-hydroxyprogesterone is extremely low, but the precursor 17-hydroxypregnenolone will be markedly elevated.
  • In 17-alpha-hydroxylase deficiency, 17-hydroxyprogesterone is extremely low, but deoxycorticosterone will be markedly elevated.
54
Q

Tests to order once the diagnosis of Turner’s syndrome has been established

A
  • Turner’s patients are at increased risk of cardiac disease, vascular disease, skeletal, renal, and hepatic disease
  • Echocardiography is the most important test to order to screen for cardiac abnormalities at the time of diagnosis
  • LFTs should be ordered regularly as part of this patient’s annual visit
55
Q

Constitutional hirsuitism

A
  • Idiopathic
  • Hirsuitism-style hair growth in the absence of high levels of circulating androgens
  • Occurs more in certain ethnic groups (Mediterranean, Indian)
56
Q

In a female-bodied, pre-pubertal child, clitoral size greater than ___ indicates clitoromegaly suggestive of virilization

A

In a female-bodied, pre-pubertal child, clitoral size greater than 1 cm indicates clitoromegaly suggestive of virilization

57
Q

The most common cause of precocious puberty is ___ and it is treated with ___.

A

The most common cause of precocious puberty is idiopathic and it is treated with GnRH agonist (leuprolide)

58
Q

Three factors vital to proper function of the endocrine system driving puberty

A
  1. Adequate body weight (input from leptin)
  2. Adequate sleep
  3. Occular exposure to sunlight
59
Q

Van Wyk Grumbach syndrome

A

When hypothyroidism causes precocious puberty

Correcting the hypothyroidism with levothyroxine causes regression of secondary sex characteristics in these patients

So, always check a TSH in patients with precocious puberty of unknown etiology!

60
Q

Telling apart gonadal dysgenesis and androgen insensitivity clinically

A
  • Gonadal dysgenesis: No estrogen OR testosterone, thus no breast development OR virilization
  • Androgen insensitivity:Breast development present (since excess testosterone is all aromatized to estrogen!)
  • Both: High risk of gonads being malignant, and both will need removal of gonads

OBGYN Clerkship (30 decks)

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