Pediatrics Repro (Dow)

Question 1

adrenarche

Answer 1

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

Question 2

thelarche

Answer 2

usually the first and most obvious sign of early puberty

this is the appearance of breast tissue

Question 3

what is the “mini puberty of infancy”

Answer 3

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

Question 4

which have earlier puberty:

caucasian or african american girls

Answer 4

African american girls

b/c of genetics and race

Question 5

when is the growth spurt in girls as compared to boys

Answer 5

two years earlier in girls

increased BMI

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

Question 6

what are some puberty related issues

Answer 6

anemia

Gynecomastia - one half of teenage boys - goes away

Acne

Psychosocial correlates of puberty

myopia
Scoliosis
STI's
Dysfunctional uterine bleeding
asthma

Question 7

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

Answer 7

testicular length more than 2.5 cm

OR

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

Question 8

stage 2 breast development

Answer 8

breast bud stage with elevation of breast and papilla

enlargement of areola

Question 9

stage 3 breast development

Answer 9

further enlargement of breast and areola

no separation of their contour

Question 10

stage 4 breast development

Answer 10

areola and papilla form a secondary mound above level of breast

Question 11

stage 5 breast development

Answer 11

mature stage

projection of papilla only

related to recession of areola

Question 12

stage 2 pubic hair development

Answer 12

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

Question 13

stage 3 pubic hair development

Answer 13

darker coarser and more curled hair

spreading sparsely

Question 14

stage 4 pubic hair development

Answer 14

hair adult in type

but covering smaller area than in adult

no spread to medial surface of thighs

Question 15

stage 2 external genitalia males

Answer 15

enlargement of scrotum and testes

Question 16

stage 3 external genitalia males

Answer 16

enlargment of penis (length and first)

further growth of testes

Question 17

stage 4 external genitalia males

Answer 17

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

testes and scrotum larger

scrotal skin darker

Question 18

is precocious puberty more common in boys or girls

Answer 18

girls

Question 19

age of precocious puberty for boys and girls

Answer 19

age 8 girls

age 9 boys

Question 20

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

Answer 20

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

Question 21

central percocious puberty

what is it?
causes?

Answer 21

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)

Question 22

peripheral percocious puberty

causes?

Answer 22

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

Question 23

incomplete percocious puberty

Answer 23

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

Question 24

premature thelarche

Answer 24

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

Question 25

premature pubarche

Answer 25

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)

Question 26

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

Answer 26

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

Question 27

central precocious puberty in girls | what are the findings?

Answer 27

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

Question 28

central precocious puberty in males | what are the findings

Answer 28

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.

Question 29

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

Answer 29

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.

Question 30

What is McCune Albright syndrome

Answer 30

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

Question 31

what on physical exam is important in evaluating for precocious puberty

Answer 31

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)

Question 32

how do you measure bone age

Answer 32

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

Question 33

how do you work up precocious puberty

Answer 33

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

Question 34

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

Answer 34

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.

Question 35

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

Answer 35

allow a child to grow to a normal adult height GnRH analogs or agonists - Leuprolide - increase final adult height resection of mass if possible

Question 36

treatment of peripheral percocious puberty

Answer 36

Does NOT respond to GnRH agonist therapy surgical managment (if tumor of testis, adrenal, ovary)

Question 37

how do you treat McCUne Albright

Answer 37

aromatase inhibitors to inhibit estrogen synthesis or block estrogen action

Question 38

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

Answer 38

hypothalamic hamartoma

Question 39

early morning vomiting and headaches?

Answer 39

tumor in head

Question 40

what is the classic triad of hypothalamic hamartoma

Answer 40

precocious puberty developmental delay seizures- laugh uncontrollably

Question 41

how do you treat idiopathic precocious puberty

Answer 41

treat with GnRH (leuprolide)- delay the puberty more common in girls

Question 42

benign thelarche

Answer 42

know this!

Question 43

effects of leuprolide

Answer 43

stopping closure of the epiphyses so they get taller