STEP 2: PEDS: CHILD DEVELOPMENT

Question 1

What are the general fields of developement?

Answer 1

gross motor, fine motor, language, social/cognitivie

Question 2

Delay in how many fields of developemnt constitutes a global developmental delay?

Answer 2

2 fields

Question 3

Primitive reflexes in kiddos indicates________________ while in adults it indicates___________________

Answer 3

Kiddos: impaired brain development and in adults a frontal lobe lesion

Question 4

What percentile do we consider head circumferance to be ‘macrocephalic’

Answer 4

97th+

Question 5

What percentile do we consider head circumferance to be ‘microcephalic’

Answer 5

3rd-

Question 6

What are our major causes of macrocephaly?

Answer 6

Hydrocephalus or tumor

Question 7

What are our major causes of microcephaly?

Answer 7

Fetal alcohol syndrome, TORCH

Question 8

What is our rule of thumb for weight gain in a neonate through 2 years of age?

Answer 8

Infants may LOSE 5-10% of their BW in first days of life but regain by 14 days, Infants DOUBLE their BW by 4-5 months, tiple it by 1 year, and quadruple it by 2 years

Question 9

At this age, an infant should be able to hold their head up when prone

Answer 9

2 months

Question 10

At this age, an infant should be able to sit unassisted

Answer 10

6 months

Question 11

Crawls, cruises, pulls to stand

Answer 11

9-11 months

Question 12

What fine motor skills do infants develop at 9-11 months

Answer 12

immature (3 finger) pincer grasp

Question 13

What fine motor skills do infants develop at 12 months?

Answer 13

mature (2 finger) pincer grasp

Question 14

When should an infant be able to build a tower of 2-4 cubes? What about 6?

Answer 14

2-4 at 18 months and 6 at 2 years

Question 15

When should an infant be able to throw a ball?

Answer 15

12 months

Question 16

When do infants start saying mama/dada?

Answer 16

9-11 months, actual first words may be at 11 months

Question 17

Uses 1-3 words and can follow one step commands

Answer 17

12 months

Question 18

Speech is 75% intelligible

Answer 18

3 years

Question 19

speech 50% intelligible

Answer 19

2 years

Question 20

This variant of normal growth is characterized by a child who often lags behind the growth curve but CONSISTENTLY lags and bone age is delayed compared to chronological age

Answer 20

Constitutional growth delay

Question 21

This variant of normal growth is characterized by a child whose growth curve shows low to normal height velocity and bone age = chronological age. Puberty is NOT delayed. Adult height remains low

Answer 21

Familial short stature

Question 22

Persistent weight for age and or length for age that is less than 5th percentule or crosses 2 major percentiles on a growth chart

Answer 22

Failure to thrive

Question 23

What are organic etiologies for FTT?

Answer 23

CF, congential heart disease, milk protein allergy, chronic infxn (HIV), hypothyroid, GERD

Question 24

What are non-organic etiologies for FTT?

Answer 24

low milk supply, inaccurate formula mixing, ppd, neglect, abuse

Question 25

What are the primitive reflexes?

Answer 25

moro, rooting, sucking, galant, palmar, plantar

Question 26

Persistence of plantar reflex after 12 months of age

Answer 26

Babinski sign, indicates UMN issue

Question 27

For infants with FTT, in what order will they fall off the curves?

Answer 27

1st weight, then length, then head circumference

Question 28

When should newborns regain their birthweight by?

Answer 28

2 weeks of life

Question 29

What is delayed puberty in males?

Answer 29

no testicular enlargement (gonadarche) by 14

Question 30

What is delayed puberty in females?

Answer 30

no thelarche by 13

Question 31

what is precoscious puberty in females?

Answer 31

signs of maturation before age 8

Question 32

what is precoscious puberty in males?

Answer 32

signs of maturation before age 9

Question 33

pathologic causes of pubertal delay

Answer 33

IBD/other systemic disease, malnutrition, gonadal dysgenesis, endocrine dysfunction (hypopit, pypothyroid, kallman, AIS, praderwilli)

Question 34

Order of puberty in females

Answer 34

thelarche, pubarche/adrenarche, growth spirt, menarche

Question 35

order of puberty in males

Answer 35

gonadarche, pubarche, adrenarche, growth spurt

Question 36

# What tanner stage: no hair in male or female, chest flat with raised nipple

Answer 36

Tanner 1

Question 37

# What tanner stage: pubic hair appears, testicular enlargement, breast buds/mounds form

Answer 37

Tanner 2 | 8-11.5 yrs ish

Question 38

# What tanner stage: Coarsening of pubic hair, penis size and length increases, breasts enlarge

Answer 38

Tanner 3 | 11.5-13 yrs ish

Question 39

# What tanner stage: coarsening of pubic hair sparing thigh, penis width/glans increases, breasts enlarge, raised areola, mound on mound

Answer 39

Tanner 4

Question 40

# What tanner stage: Coarse hair across pubis and medial thigh, penis and testes enlare to adult size, aereola flattens

Answer 40

tanner 5

Question 41

X linked mutation leading to a genotypic male with phenotype of female. Breast development, absent or minimal axillary and pubic hair. NO uterus, cervix. Primary amenorrhea.

Answer 41

AIS ## Footnote these pts need an elective gonadectomy due to cancer risk

Question 42

What is our first step in assessing primary amenorrhea?

Answer 42

Do they have a uterus???

Question 43

If a pt exhibits primary amenorrhea and an absent uterus, what is on your diff?

Answer 43

AIS, XX mullerian agenesis ## Footnote Note that swyer syndrome (XY dysgenesis) they HAVE a uterus

Question 44

In the assesment of primary amenorrhea, if they have a uterus, what is the next step?

Answer 44

FSH

Question 45

In the assesment of primary amenorrhea and they have a uterus and you do an fsh and find that it is HIGH, what is on your diff?

Answer 45

HIGH FSH should prompt you do think of doing a karyotype anaylsis. This will help you differentiate TURNER from primary ovarian insufficiency!

Question 46

Primary amenorrhea + uterus present + normal FSH should make you think....

Answer 46

imperforate hymen

Question 47

Primary amenorrhea + present uterus + low FSH should make you think....

Answer 47

Pit-thalam-issue of some kind. Get a TSH and prolactin to help you diff hypothyroidism, prolactinoma, and functional hypothalamic amenorrhea

Question 48

the absence of menarche in a girl greater than or equal to 13 with NO secondary sexual characteristics

Answer 48

Primary Amenorrhea

Question 49

In the evaluation of precocious puberty, what is your first test?

Answer 49

Wrist xray for bone age

Question 50

A child exhibiting signs of secondary sex characterstics with normal bone age. They only are demonstrating pubic hair development without other changes. What is the likely culprit?

Answer 50

Premature adrenarche

Question 51

If you are assesing someone for precocious puberty and they exhibit advanced bone age, what is your next move?

Answer 51

LH

Question 52

Precocious puberty assesment--> advanced bone age--> high basal LH--> what should you be thinking?

Answer 52

central precosious puberty (idiopathic, cns tumor)

Question 53

In a patient being evaluated for precocious puberty and they have advanced bone age, high basal LH or high LH w/ a GnRH stim test, what should be your next move?

Answer 53

Head MRI for hypothalamic or pituitary tumor

Question 54

What is our mainstay of treatment for idiopathic central precocious puberty?

Answer 54

GnRH agonist ## Footnote prevents premature epiphyseal plate fusion and maximizes adult height potential. leuprolide, buserelin, goserelin

Question 55

The premature onset of secondary sexual characteristics due to increased gonadotropin-releasing hormone secretion. Typically caused by idiopathic activation of the hypothalamic-pituitary-gonadal axis; can be caused by CNS tumors.

Answer 55

Central precocious puberty Gonadotropin-dependent precocious puberty

Question 56

No increase in LH levels after GnRH administration is characterstic of what kind of precocious puberty?

Answer 56

Peripheral precocious puberty

Question 57

What are some etiologies for peripheral precocious puberty?

Answer 57

Increased androgen production (CAH, sertoli-leydig cell tumor, leydig cell tumor), Increased estrogen production (McCune-Albright, dysgerminoma), elevated beta HCG production (dysgerminoma, embryonal cell carcinoma, choriocarcinoma), primary hypothyroid, exogenous steroid use, obesity related

Question 58

Mosaic mutation in the GNAS1 gene on chromosome 20 (autosomal recessive inheritance) Some cells have a normal version of the GNAS1 gene, while other cells have the mutated version. Embryos only survive if mosaicism occurs. A genetic syndrome caused by a G-protein activating mutation and subsequent continuous stimulation of endocrine functions

Answer 58

McCune-Albright

Question 59

What are the clinical features of McCune albright?

Answer 59

Unilateral cafe au lait, polyostotic fibrous dysplasia, peripheral precocious puberty, cushing, acromegaly, hyperthyroid ## Footnote The 3 P's of McCune-Albright syndrome are Polyostotic fibrous dysplasia, Pigmentation (café-au-lait spots), and Precocious puberty.

Question 60

Klinefelter, turner, and AIS are all considered

Answer 60

primary gonadal insufficiency in hypergonadotropic hypogonadism

Question 61

In patients with delayed puberty, what might the following labs tell you: serum prolactin IGF-1 Karyotype Antiendomysial antibody Ab US Head MRI

Answer 61

serum prolactin: prolactinoma? IGF-1: growth hormone def Karyotype: turner/klinefelter Antiendomysial antibody:celiac + malabsorp Ab US: streak ovaries, testicular mass Head MRI: prolactinoma

Question 62

Primary treatment for constitutional growth delay

Answer 62

expectant managemnt