Abnormal peds growth

Question 1

T or F: normal growth is pulsatile

Answer 1

true

Question 2

T or F: normal growth is seasonal

Answer 2

true

Question 3

normal portionality for weight-to-height

Answer 3

2.3rd-97.7th percentile

Question 4

body proportion (US/LS) is higher or lower in turners?

Answer 4

incr (longer US, shorter LS)

Question 5

body proportion (US/LS) is higher or lower in marfan?

Answer 5

decr

Question 6

FTT is based on decreased velocity of Weight or Length

Answer 6

WEIGHT

Question 7

FTT is defined by (2)

Answer 7

1. weight under 2nd percentile

2. decr velocity of weight gain

Question 8

how to determine if a child’s growth is abnormally rapid

Answer 8

height-for-age curve deviated upwards across 2 major height % curves

Question 9

what can you evaluate if there is evidence of accelerated growth

Answer 9

bone age

Question 10

advanced bone age is ____% above chronological age

Answer 10

20%

Question 11

most common overgrowth in infancy is

Answer 11

maternal diabetes

Question 12

macrocephaly, frontal bossing, hypertelorism, intellectual disability, large at birth, advanced bone age, IFG1 normal

Answer 12

cerebral gigantism (sotos syndrome)

Question 13

advanced bone age, rapid growth then slows, macrosomia, visceromegaly, macroglossia, omphalocele, hypoglycemia, hyperinsulinism, renal issues

Answer 13

beckwith-wiedemann syndrome

Question 14

most common cause of tall state

Answer 14

familial tall stature

Question 15

precocious puberty is more common in girls or boys

Answer 15

girls

Question 16

types of precocious puberty

Answer 16

Central GDPP

peripheral GDIP

Question 17

those with precocious puberty have ___bone age, ___epihpyseal closure

Answer 17

advanced bone age, early epiphyseal closure

Question 18

most reliable measurement of growth hormone excess

Answer 18

IGFBP-3

Question 19

long legs, reduced UL segment ratio, low sitting height (eunuchoid proportions)

Answer 19

sex hormone deficiency

Question 20

mutation of ACTH receptor is primary or secondary adrenal insufficiency

Answer 20

primary adrenal insufficiency (high ACTH, low cortisol)

Question 21

tall, obese, early onset puberty, low GH but normal IGF

Answer 21

exogenous obesity

Question 22

what is the most common monogenic cause of human obesity?

Answer 22

melanocortin 4 receptor (MC4R)

Question 23

hyperphagia, hyperinsulinemia, obese kid, preserved reproductive fxn

Answer 23

MC4R mutation

Question 24

47XXY, long legs, short trunk, small testes, gynecomastia, learning disabled, bone age normal/delayed

Answer 24

klinefelter

Question 25

how to tx klinefelter

Answer 25

testosterone

Question 26

delayed motor/ language development, large teeth, tremor, ADHD, autism

Answer 26

47 XYY syndrome

Question 27

lens subluxated upward

Answer 27

marfan

Question 28

lens subluxated downward

Answer 28

homocystinuria

Question 29

does marfans or homocysteinuria have risk for thrombotic events?

Answer 29

homocysteinuria

Question 30

does marfans or homocysteinuria have risk for intellectual disability?

Answer 30

homocysteinuria

Question 31

tall, AD, dysregulated GH, precocious pubterty, cafe au lait spots, hamartomas, gliomas

Answer 31

von recklinghausen disease (NF1)

Question 32

madelung deformity of the forearm

Answer 32

turner syndrome

Question 33

if you see a girl with short stature, what should be in your differential?

Answer 33

turner syndrome

Question 34

obesity, severe hyperphagia, developmental delay, hypogonadism

Answer 34

prader-willi

Question 35

how to tx prader-wili

Answer 35

GH

Question 36

hypertelorism, downward slanting eyes, low ears, pectus excavatum, webbed neck, intellectual disability

Answer 36

noonan syndrome

Question 37

how to tx noonan syndrome

Answer 37

GH

Question 38

short trunk dwarfism, mostly affects spine

Answer 38

spondyloepiphyseal dysplasia