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medicine > Puberty and its abberrations > Flashcards

Puberty and its abberrations Flashcards

1
Q

What neurons acts as central processors for relaying signals from the periphery to GnRH neurons?

A

Kisspeptin neurons

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2
Q

What is pubarche?

A

presence of pubic hair

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3
Q

What is adrenarche?

A

presence of androgen dependent changes including pubic hair, apocrine body odor, acne, body hair

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4
Q

What is gonadarche?

A

earliest changes of gonads due to gonadotropin stimulation

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5
Q

What is thelarche?

A

presence of estrogen dependent breast tissue

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6
Q

What is menarche?

A

onset of menses

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7
Q

What is gynecomastia?

A

enlargement of male breast tissue resultant of imbalance of hormones

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8
Q

How is puberty defined in females?

A

presence of pubarche with thelarche

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9
Q

How is puberty defined in males?

A

presence of pubarche with gonadarche

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10
Q

What age defines precocious puberty in boys?

A

Before 9 years of age

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11
Q

What age is normal pubertal onset for boys and girls?

A

1) girls: 8-13

2) boys: 9-14

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12
Q

What hormones signify gonadarche?

A

1) in boys - testosterone

2) in girls - estrogen

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13
Q

What hormones signify adrenarche?

A

adrenal androgens (testosterone, DHEAS, androstenedione)

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14
Q

What hormone influences growth plate fusion?

A

estrogen in both sexes

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15
Q

What non-invasive test can be used to further define pubertal advancement?

A

bone age

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16
Q

What are the risks for precocious puberty?

A

1) early maternal menarche
2) low birth weight
3) excessive weight gain or obesity in infancy
4) after international adoption
5) after intercranial radiation

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17
Q

Is isolated FSH elevation idicative of precocious puberty?

A

No - FSH spikes with GnRH occur even in prepuberty states

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18
Q

What findings will you have gonadotropin-dependent puberty? (true puberty)

A

1) secondary sexual characteristics
2) bone age advancement
3) accelerated linear growth
4) gonadal maturation
5) gonadotropins (LH/FSH) - pubertal baseline and stimulated
6) sex steroids - pubertal

19
Q

What findings will you have in gonadotropin independent puberty? (pseudoprecocity)

A

1) secondary sexual characteristics
2) bone age advancement
3) accelerated linear growth
4) None/minimal gonadal maturation
5) prepubertal gonadotropins (LH/FSH)
6) sex steroids - pubertal

20
Q

What are some symptoms of McCune Albright Syndrome?

A

precocious puberty, cafe au lait pigmentation (coast of maine), polyostotic fibrous dysplasia

21
Q

What mutation is found in McCune Albright syndrome?

A

activating mutation of the GNAS1 gene which encodes the alpha subunit of the G protein

22
Q

What is testitoxicosis?

A

familial or sporadic male-limited sexual precocity

23
Q

What mutation is found in testitoxicosis?

A

constitutive activation of mutant LH receptors (autosomal dominant or sporadic)

24
Q

What are the signs/symptoms of testitoxicosis?

A

precocious puberty early in childhood (often by 2-3 years of age) , high testosterone, suppressed gonadotropins (testicular volume is less than expected for degree of sexual development)

25
Q

What mutation is found in Albright’s hereditary osteodystrophy?

A

GNAS activating mutation that encodes for a Galpha subunit

26
Q

How does temperature affect the mutation in albright’s?

A

1) at lower temperature (in gonads) gain of function - gonadotropin independent precocious puberty
2) at normal body temperature - loss of function (pseudohypoparathyroidism)

27
Q

What is hCG?

A

human chorionic gonadotropin - an LH receptor agonist (high levels stimulate LH receptor thereby promoting androgen production)

28
Q

What does hCG mediated sexual precocity rarely occur in girls?

A

both LH and FSH are needed for ovarian stimulation

29
Q

What is the only form of sexual precocity with growth arrest?

A

Van Wyk-Grumbach Syndrome

30
Q

What is Van Wyk-Grumbach syndrome?

A

sexual precocity in association with hypothroidism (often associated with galactorrhea, multiple ovarian cysts, little/no pubarche)

31
Q

What can testosterone exposure cause?

A

isolated pubarche

32
Q

What can estrogen exposure cause?

A

1) isolated thelarche in boys and girls

2) menstrual bleeding in girls

33
Q

What defines delayed puberty?

A

lack of secondary sexual findings in boys by age 14 and age 12 or 13 in girls (lack of menarche by age 15-16 in girls)

34
Q

What describes constitutional delay of growth and puberty?

A

1) short stature AND delayed puberty
2) see fall in growth % over first two years of life followed by normal pre-pubertal growth velocity and then delayed puberty

35
Q

What is the differential for someone with normal growth, slight bone age delay and poor weight?

A

1) eating disorder
2) nutritional problem
3) competitive athlete

36
Q

What is the differential for someone with normal growth, slight bone age delay and normal weight?

A

1) if LH/FSH is high - hypergonadotropic hypogonadism
2) prepubertal LH/FSH levels - if prolactin is normal (hypogonadotropic hypogonadism), if prolactin is elevated (prolactinoma or CNS mass)

37
Q

What is the DD for decreased growth velocity, fairly normal bone age and elevated weight?

A

1) if prepubertal LH/FSH with normal thyroid function - cushing’s
2) prepubertal LH/FSH with elevated TSH - hypothyroidism

38
Q

What is the DD for decreased growth velocity, fairly normal bone age and normal weight?

A

1) elevated LH/FSH and normal IGF-1 - Turners
2) prepubertal LH/FSH, low IGF-1
a) normal T4 and prolactin = isolated GH
deficiency
b) low T4, variable prolactin = consider
hypopituitarism

39
Q

What is Kallman syndrome?

A

X-linked recessive form of hypogonadotropin hypogonadism associated with anosmia due to failed migration of GnRH neurons from the olfactory placode into the forebrain

40
Q

What mutations are found in Kallman syndrome?

A

mutations in the KAL gene of Xp22.3

41
Q

What is klinefelter syndrome?

A

the most common cause of male hypogonadism and infertility

42
Q

What is the clinical course of klinefelters?

A

1) enter puberty normally but testosterone levels fall in late adolescence and early adulthood
2) some academic delays
3) long extremities
4) gynecomastia

43
Q

When should you consider Turner Syndrome in your DD?

A

with short stature and ovarian dysfunction

44
Q

What is the clinical course of Turners?

A

1) normal statural growth for 1st 2-3 years followed by deceleration and typically normal bone age
2) 1/3 enter puberty normally

medicine (15 decks)

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