HYHO- Neman #1

Question 1

a single point on the growth chart only gives you information about how they plot on the curve on?

Answer 1

on THAT day

Question 2

_ _ are essential to evaluare the growth pattern to determine if the rate of growth is adequate

Answer 2

multiple points

Question 3

normal BMI

Answer 3

18.5-24.9

Question 4

what is the normal growth velocity for school aged kids? - age 5-puberty

Answer 4

5cm/year or 2 inches/year

linear growth

Question 5

what is the growth parameter most commonly first affected in children with endocrine growth disorders?

Answer 5

length or heigh

Question 6

what is delayed bone age

Answer 6

this is a bone age that is 2 standard deviations or more below the chronological age of the patient

Question 7

be concered when points on a growth chart begin to _ up or down from what had been a normal growth rate for that child

Answer 7

deviate

look for deviations up or down

Question 8

what are the growth parameters on a growth chart

Answer 8

height (length if less than 24 months)
weight
BMI (if greater than 36 months)
head circumference (if less than 24 months)

Question 9

if height/length changes first or most drastically on the growth chart think

Answer 9

endocrine- growth hormone def

Question 10

if weight changes first or most drastically on the growth chart think

Answer 10

calories/nutrition

Question 11

if the head circumference changes first or most drastically on the growth chart think

Answer 11

brain/skull/hydrocephalus

Question 12

what is short stature

Answer 12

height greater than 2 standard deviations below the mean height (50th percentile) for age and sex

less than the 3rd percentile on the growth chart

Question 13

what are the 3 benign causes of short statue

Answer 13

familial - parents are small

constiutional

idiopathis

Question 14

in familial causes of short stature the kid grows at a normal velocity in all parameters but jsut plot lower for height; end height is consistent with mid-parental ht.

is bone age consistent with chronological age?

Answer 14

yes

Question 15

what is the cause of constiutional short stature

Answer 15

the kid grows at a low normal rate and plot low on the growth curve

puberty is delayed “late bloomers”

end hight is normal and groth spurts occur later

Question 16

the hallmark of constiutional delay is ?

Answer 16

delyaed bone age

Question 17

what is idopathic short stature

Answer 17

short stature that is not caused by endocrine, metabolic, or other diagnosis, and there is no family hisotry of short stature

bone age is consistent with chronilogical age

Question 18

what is important to workup in a kid with short stature?

Answer 18

IGF-1 (insulin like growth factor) and IGFBP-3 (igf binding protein 3)

Question 19

igf-1 is?

Answer 19

this is a hormone that functions as the major mediator of growth hormone stimulated somatic growth

Question 20

IGFBP-3 is?

Answer 20

the main IGF-1 transport protein in the blood stream

Question 21

_ increases IGF-1 synthesis by _ and _ suggetive that is contributes to statural growth

Answer 21

growth hormone

osteoblasts and chondrocytes

Question 22

in a patient with short stature there are decreased levels of?

Answer 22

IGF-1 and IGFBP-3 that are consistent with growth hormone defecinecy

Question 23

growth hormone secretion is _ while IGF-1 and IGFBP-3 levels are _

Answer 23

pulsatile

stable (meaning they can be drawn at any time of day)

Question 24

if the levels of IGF-1 and IGFBP-3 are decreases growth hormone stimulation must be tested and what are the three most reliable tests

Answer 24

adminsitration of glucagon
administration of arginine
insulin induced hypoglycemia

Question 25

precocoius puberty is defined as?

Answer 25

onset of secondary sexual characterisits in girls before 8 and in boys before 9

Question 26

the diagnosis of precoccious puberty should be entertained when girls: boys: overall:

Answer 26

girls: if they have progressive breast development and crossing major percentile lines upward on the linear growth chart boysL testicular and penile enlargment crossing major percentile lines upward on the linear growth chart overall: if the rate of growth is fast initially but overall end-heigh is less than it would normally be

Question 27

workup for percoccious puberty

Answer 27

1. H and P 2. labortory tests 3. imaging

Question 28

what labs should you get in precoccious puberty

Answer 28

LH, FHS, Estradiol and testosterone, 17-hydroxyprogesterone

Question 29

what imaging do we get for precoccious puberty

Answer 29

bone age (advanced) MRI of the head (CNS tumor?) ultrasound of the gonads

Question 30

what are the karyotypes of tuner syndrome

Answer 30

45X0- 60% of cases 45X/46XX and other mosaicisms - 15% of cases 25% of casses have 2 X chromosomes but the short arm of one X chromosome is missing

Question 31

# most ppl with turner syndrome die in utero -98% features of turner syndrome

Answer 31

short stature, wide shaped chest, wide spaced hypoplastic nipples, excessive skin at the back of the neck (webbed neck), gonadal dysgenesis , horsehose kidney , congenital heart disease ## Footnote streak ovaries, primary amenorrhea, lack of pubertal development

Question 32

what is premature adrenarch

Answer 32

development of coarse dark pubic hair before 8 in girls and before 9 in boys

Question 33

premautre adrenarch may be associated with?

Answer 33

body odor, acne, increased oliness of hair and skin

Question 34

what causes premature adrenarche

Answer 34

a benign condition that caused by early maturation of the zona reticularis of the adrenal gland that is associated with increased androgen secretion

Question 35

when should you workup premature adrenarche

Answer 35

bone age assessment should be done: if normal or slightly advanced then it is most likely bening premature adrenarche follow them clincially IF it is associated with other secondary characterisitcs or bone age is advanced greater than 2 SD from the normal age---> workup - clitoral enlargement, breast enlargement, penile enlargement, testicular enlargement IF associated with significant growth acceleration

Question 36

whether premature adrenarch is benign or associated with precoicous puberty the patient needs to be?

Answer 36

closely monitored clinically (yearly)

Question 37

what is premature thelarche

Answer 37

breast development before age 8 can start as early as 6 months most almost always associated with a normal growth rate

Question 38

when is work up for premature thelarche warranted

Answer 38

when associated with other signs of secondary sex maturation, if there is accelerated growth, if bone age is advanced before 2 SD for age

Question 39

congenital hypothyroidism in kids affects _ more, it is the inaquadte production of?

Answer 39

females (2:1) thyroid hormone production

Question 40

congential hypothyroidism in kids is caused by?

Answer 40

absnece, dygenesis, or abnormal functioning og the thyroid gland

Question 41

what is the most common preventable causes of intellectual disability in children world wide

Answer 41

congential hypothyroidism (the later treatment is initiated the lower the final IQ)

Question 42

signs of congenital hypothyroidism in kids

Answer 42

can be asymptomatic signs can develop over the first few months of life: hoards cry, coarse facies, lethargy, feeding problems, constipation, macroglossia, hypotonia, large fontanels, prolonged jaundice ## Footnote there is a newborn metabolic screen to check for this

Question 43

what is the most common cause of acquired hypothyroidism?

Answer 43

autoimmune thyroiditis

Question 44

what are the clinical manifestations of acquired hypothyroidism

Answer 44

declining growth velocity/short stature abnormal pubertal development (delay) declining school preformance sluggishness, fatigue, cold intolerance, constipation, dry skin, brittle hair, facial puffiness, muscle aches and pains

Question 45

linear growth depends on the balance between _ and _ of the _ at the growth plate

Answer 45

proliferation and deterioration of the chondrocytes at the growth plate

Question 46

what is the primary determinant of normal post natal growth and skeletal maturation

Answer 46

T3 (thyroid hormone)

Question 47

what is an important regulator of bone and mineral metabolism in older children and adults

Answer 47

T3 (thyroid hormone)

Question 48

what is a major prerequisite of normal matruation of bone?

Answer 48

T3 (thyroid hormone)