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Repro II Exam II > Pediatrics Repro (Dow) > Flashcards

Pediatrics Repro (Dow) Flashcards

1
Q

adrenarche

A

Adrenarche is the activation of the adrenal cortex for the production of adrenal androgens, and typically occurs before the onset of puberty.
Pubic and axillary hair may appear before, at about the same time, or well after the appearance of breast tissue

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2
Q

thelarche

A

usually the first and most obvious sign of early puberty

this is the appearance of breast tissue

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3
Q

what is the “mini puberty of infancy”

A

the hypothalamic-pituitary-gonadal axis is biologically active in infancy with peak activity between six and eight weeks of age. This state yields sex steroid levels comparable with those seen in early-to-mid puberty, but without peripheral effects.

Hormonal changes after birth: falling levels of maternal estrogens
65-90% of neonates have breast tissue
Vaginal discharge can be observed.
“Witches’ milk” from breasts and even menses can be seen
Infantile acne also common

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4
Q

which have earlier puberty:

caucasian or african american girls

A

African american girls

b/c of genetics and race

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5
Q

when is the growth spurt in girls as compared to boys

A

two years earlier in girls

increased BMI

  • early due to lean body mass
  • later due to fat
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6
Q

what are some puberty related issues

A

anemia

Gynecomastia - one half of teenage boys - goes away

Acne

Psychosocial correlates of puberty

myopia
Scoliosis
STI's
Dysfunctional uterine bleeding
asthma
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7
Q

in male genitalia, what are the early signs of puberty?

A

testicular length more than 2.5 cm

OR

testicular volume (with Prader orchidometer beads) is 4 mL or more

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8
Q

stage 2 breast development

A

breast bud stage with elevation of breast and papilla

enlargement of areola

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9
Q

stage 3 breast development

A

further enlargement of breast and areola

no separation of their contour

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10
Q

stage 4 breast development

A

areola and papilla form a secondary mound above level of breast

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11
Q

stage 5 breast development

A

mature stage

projection of papilla only

related to recession of areola

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12
Q

stage 2 pubic hair development

A

sparse growth of long slightly pigmented hair straight or curled along labia

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13
Q

stage 3 pubic hair development

A

darker coarser and more curled hair

spreading sparsely

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14
Q

stage 4 pubic hair development

A

hair adult in type

but covering smaller area than in adult

no spread to medial surface of thighs

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15
Q

stage 2 external genitalia males

A

enlargement of scrotum and testes

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16
Q

stage 3 external genitalia males

A

enlargment of penis (length and first)

further growth of testes

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17
Q

stage 4 external genitalia males

A

increased size of penis with growth in breadth and development of glans

testes and scrotum larger

scrotal skin darker

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18
Q

is precocious puberty more common in boys or girls

A

girls

19
Q

age of precocious puberty for boys and girls

A

age 8 girls

age 9 boys

20
Q

early growth spurt in precocious puberty causes tall stature but rapid bone maturation. why is this a problem?

A

can cause premature and rapid skeletal maturation and fusion of the epiphyses –> can cause linear growth to cease too early and can result in short adult stature

evaluate with BONE AGE

21
Q

central percocious puberty

what is it?
causes?

A

early matureation of the entire hypothalamic pituitary gonadal axis. Gonadotropin dependent
with the full spectrum of physical and hormonal changes of puberty

Causes:

1) idiopathic most of the time > 80 percent (a diagnosis of exclusion)
- girls you want to just slow their puberty down a bit
- boys you want to explore for a tumor

2) CNS abnormalities:
- Hypothalamic hamartomas (secrete GnRH)
- Tumors
- Acquired CNS injury
- congenital anomalies

3) Previous excess sex steroid exposure (rare)

22
Q

peripheral percocious puberty

causes?

A

gonadotropin independent

1/5 th as common as central precocious puberty

peripheral secretions of sex hormones from:
Gonads
-ovarian cysts or tumors - most common cause in girls

Male limited syndromes
(FMPP) - familial male precocious puberty

Adrenal glands

  • tumor
  • CAH
  • ectopic HCG by germ cell tumor

Exogenous sources of sex steroids
-possibly soy

McCune Albright

23
Q

incomplete percocious puberty

A

Premature pubarche and premature thelarche are two common, benign, normal variant conditions that can resemble precocious puberty but are nonprogressive or very slowly progressive.

24
Q

premature thelarche

A

Premature thelarche refers to the isolated appearance of breast development, usually in girls younger than 3 years;

25
Q

premature pubarche

A

premature pubarche refers to appearance of pubic hair without other signs of puberty in girls or boys younger than 7-8 years (usually progression is slow, so ultimate height is okay)

26
Q

how do you tell the difference b/w peripheral and central percocious puberty

A

measure LH levels

In central precocious puberty (GDPP), basal LH levels are often elevated into the pubertal range and show a pubertal (heightened) response to GnRH stimulation.

In peripheral precocious puberty (GIPP), the LH level is low at baseline and fails to respond to GnRH stimulation

27
Q

central precocious puberty in girls

what are the findings?

A

breast enlargement

  • usually the first and most obvious sign of early puberty
  • due to increased estrogen production

Pubic and axillary hair may appear before, at about the same time, or well after the appearance of breast tissue

axillary odor
menarche - 2-3 years after breast development

enlargement of the clitoris is androgen excess and must be investigated

investigate severe acne

28
Q

central precocious puberty in males

what are the findings

A

The earliest sign of central precocious puberty (CPP) is enlargement of the testes, which depends on increased production of follicle-stimulating hormone (FSH); testicular length is more than 2.5 cm or testicular volume (with Prader orchidometer beads) is 4 mL or more
subtle finding that often goes unnoticed by patients and parents

Other signs of puberty (eg, penis growth, reddening and thinning of the scrotum, increased pubic hair) are a consequence of increased testosterone production and occur within 1-2 years after testicular enlargement.

Accelerated linear growth (the pubertal growth spurt) occurs later in the course of male puberty than in female puberty but often occurs by the time other physical changes are noted.

29
Q

what is the meaning of pubic hair that grows without penis and testicular enlargement and other signs of increased androgen produciton

A

Pubic hair growth that occurs without penis and testicular enlargement and other signs of increased androgen production is less likely true puberty - eg, premature adrenarche or a mild, nonclassic form of congenital adrenal hyperplasia.

30
Q

What is McCune Albright syndrome

A

has 2 of the following features:

Polyostotic fibrous dysplasia

  • many fx’s early
  • gait anomalies, bony deformities

Cafe au lait skin

autonomous endocrine hyperfunction

  • peripheral percocious puberty (more in girls)
  • *** they sometimes have menarche first

other endocrine syndromes

  • hyperthyroid
  • acromegaly
  • Cushings
31
Q

what on physical exam is important in evaluating for precocious puberty

A

growth velocity

androgenic effects:
-acne, hirsutism, increased muscle mass and clitoromegaly ***

estrogenic effects:
-breast development and changes in vaginal mucosa (more pink and thickened with puberty)

32
Q

how do you measure bone age

A

radiograph of the nondominant hand and wrist

Vital in the evaluation of sexual precocity; always advanced for chronologic age in cases of pathologic precocious puberty
to distinguish between benign early adrenarche and precocious puberty
Helps determine whether treatment to stop additional development will be of value

33
Q

how do you work up precocious puberty

A

bone age **

LH levels ***

  • high in central with a heightened response to GnRH stimulation
  • low in peripheral and fail to respond to GnRH stimulation

Testosterone, estradiol, LH, FSH, cortisol, DHEA, 17-hydroxyprogesterone

HCG in boys

34
Q

in boys that you suspect have precocious puberty, what must you perform in the workup

A

MRI of the head

The younger the child with central precocious puberty, the greater the chance of finding CNS pathology (among children younger than 6 years).

For boys younger than 9 years, the incidence of CNS findings is much higher than in girls, and MRI should be part of the evaluation.

35
Q

what is the primary goal of treatment for GDPP (central)

A

allow a child to grow to a normal adult height

GnRH analogs or agonists

  • Leuprolide
  • increase final adult height

resection of mass if possible

36
Q

treatment of peripheral percocious puberty

A

Does NOT respond to GnRH agonist therapy

surgical managment (if tumor of testis, adrenal, ovary)

37
Q

how do you treat McCUne Albright

A

aromatase inhibitors to inhibit estrogen synthesis or block estrogen action

38
Q

what is the most frequent type of CNS tumor to cause precocious puberty in very young children

A

hypothalamic hamartoma

39
Q

early morning vomiting and headaches?

A

tumor in head

40
Q

what is the classic triad of hypothalamic hamartoma

A

precocious puberty
developmental delay
seizures- laugh uncontrollably

41
Q

how do you treat idiopathic precocious puberty

A

treat with GnRH (leuprolide)- delay the puberty

more common in girls

42
Q

benign thelarche

A

know this!

43
Q

effects of leuprolide

A

stopping closure of the epiphyses so they get taller

Repro II Exam II (8 decks)

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