HYHO- Neman #1 Flashcards
a single point on the growth chart only gives you information about how they plot on the curve on?
on THAT day
_ _ are essential to evaluare the growth pattern to determine if the rate of growth is adequate
multiple points
normal BMI
18.5-24.9
what is the normal growth velocity for school aged kids? - age 5-puberty
5cm/year or 2 inches/year
linear growth
what is the growth parameter most commonly first affected in children with endocrine growth disorders?
length or heigh
what is delayed bone age
this is a bone age that is 2 standard deviations or more below the chronological age of the patient
be concered when points on a growth chart begin to _ up or down from what had been a normal growth rate for that child
deviate
look for deviations up or down
what are the growth parameters on a growth chart
height (length if less than 24 months)
weight
BMI (if greater than 36 months)
head circumference (if less than 24 months)
if height/length changes first or most drastically on the growth chart think
endocrine- growth hormone def
if weight changes first or most drastically on the growth chart think
calories/nutrition
if the head circumference changes first or most drastically on the growth chart think
brain/skull/hydrocephalus
what is short stature
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
what are the 3 benign causes of short statue
familial - parents are small
constiutional
idiopathis
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?
yes
what is the cause of constiutional short stature
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
the hallmark of constiutional delay is ?
delyaed bone age
what is idopathic short stature
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
what is important to workup in a kid with short stature?
IGF-1 (insulin like growth factor) and IGFBP-3 (igf binding protein 3)
igf-1 is?
this is a hormone that functions as the major mediator of growth hormone stimulated somatic growth
IGFBP-3 is?
the main IGF-1 transport protein in the blood stream
_ increases IGF-1 synthesis by _ and _ suggetive that is contributes to statural growth
growth hormone
osteoblasts and chondrocytes
in a patient with short stature there are decreased levels of?
IGF-1 and IGFBP-3 that are consistent with growth hormone defecinecy
growth hormone secretion is _ while IGF-1 and IGFBP-3 levels are _
pulsatile
stable (meaning they can be drawn at any time of day)
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
adminsitration of glucagon
administration of arginine
insulin induced hypoglycemia
precocoius puberty is defined as?
onset of secondary sexual characterisits in girls before 8 and in boys before 9
the diagnosis of precoccious puberty should be entertained when
girls:
boys:
overall:
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
workup for percoccious puberty
- H and P
- labortory tests
- imaging
what labs should you get in precoccious puberty
LH, FHS, Estradiol and testosterone, 17-hydroxyprogesterone
what imaging do we get for precoccious puberty
bone age (advanced)
MRI of the head (CNS tumor?)
ultrasound of the gonads
what are the karyotypes of tuner syndrome
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
most ppl with turner syndrome die in utero -98%
features of turner syndrome
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
streak ovaries, primary amenorrhea, lack of pubertal development
what is premature adrenarch
development of coarse dark pubic hair before 8 in girls and before 9 in boys
premautre adrenarch may be associated with?
body odor, acne, increased oliness of hair and skin
what causes premature adrenarche
a benign condition that caused by early maturation of the zona reticularis of the adrenal gland that is associated with increased androgen secretion
when should you workup premature adrenarche
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
whether premature adrenarch is benign or associated with precoicous puberty the patient needs to be?
closely monitored clinically (yearly)
what is premature thelarche
breast development before age 8
can start as early as 6 months
most almost always associated with a normal growth rate
when is work up for premature thelarche warranted
when associated with other signs of secondary sex maturation, if there is accelerated growth, if bone age is advanced before 2 SD for age
congenital hypothyroidism in kids affects _ more, it is the inaquadte production of?
females (2:1)
thyroid hormone production
congential hypothyroidism in kids is caused by?
absnece, dygenesis, or abnormal functioning og the thyroid gland
what is the most common preventable causes of intellectual disability in children world wide
congential hypothyroidism
(the later treatment is initiated the lower the final IQ)
signs of congenital hypothyroidism in kids
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
there is a newborn metabolic screen to check for this
what is the most common cause of acquired hypothyroidism?
autoimmune thyroiditis
what are the clinical manifestations of acquired hypothyroidism
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
linear growth depends on the balance between _ and _ of the _ at the growth plate
proliferation and deterioration of the chondrocytes at the growth plate
what is the primary determinant of normal post natal growth and skeletal maturation
T3 (thyroid hormone)
what is an important regulator of bone and mineral metabolism in older children and adults
T3 (thyroid hormone)
what is a major prerequisite of normal matruation of bone?
T3 (thyroid hormone)