Peds Endo & Clinical Adrenal (Newman)

Question 1

Information garnered by a single point on the growth chart?

Answer 1

single points gives information about how they plot for THAT day; multiple points are needed to evaluate if the rate of growth is adequate

Question 2

How many points are needed to gather if the rate of growth on a growth chart is adequate?

Answer 2

multiple

Question 3

Normal range for a BMI

Answer 3

18.5 - 24.9

Question 4

Normal growth velocity for school age children

Answer 4

linear growth; 5cm/year (2 inches/yr)

Question 5

the most common first affected growth parameter in children with endocrine growth disorders

Answer 5

length or height

Question 6

delayed bone age

Answer 6

bone age that is 2 standard deviations or more below the chronological age

Question 7

If height changes first, what should you think?

Answer 7

endocrine problem

Question 8

If weight changes first, what should you think?

Answer 8

calorie/nutrition problem

Question 9

If the head circumference changes first, what should you think?

Answer 9

brain, skull or hydrocephalus

Question 10

What are the 3 common benign causes of short stature?

Answer 10

1. familial - parents are small
2. constitutional - puberty is delayed
3. idiopathic - we don’t know

Question 11

IGF-1 and IGF Binding Protein in regards to short stature

Answer 11

GH activates IGF-1, IGF-1 is transported in blood by binding protein, travels to bone, and initiates osteoblasts and chondrocytes to contribute to growth

Question 12

Low levels of what is consistent with a GH deficiency in a patient with short stature?

Answer 12

IGF-1 and IGFBP-3

Question 13

What are the 3 most reliable test for a patient with short stature who has GH deficiency?

Answer 13

1. Administration of glucagon
2. Administration of arginine
3. Insulin-induced hypoglycemia

Question 14

Definition of precocious puberty

Answer 14

before 8 in girls
before 9 in boys

Question 15

Workup for suspected precocious puberty?

Answer 15

Complete H&P
Lab tests: LH, FSH, and estradiol/testosterone
Imaging: Bone age (will be advanced)

Question 16

Turner’s syndrome

Answer 16

45 XO; short stature, wide shield-shaped chest, wide-spaced hypoplastic nipples, webbed neck, “streak ovaries”, primary amenorrhea

associated w/ horseshoe kidneys and congenital heart disease

Question 17

Premature adrenarche

Answer 17

premature pubic/axillary hair
may be associated with body odor, acne and increased oiliness; early maturation of zona reticularis (androgens); pt needs to be monitored closely (yearly)

Question 18

Premature thelarche

Answer 18

premature breast development
before the age of 8 in females; may start as early as 6 months of age; work-up is warranted when associated w other secondary sex maturation

Question 19

Hypothyroidism in children

Answer 19

females; intellectual disability (low IQ); hoarse cry, coarse facies, lethargy, feeding problems, constipation and macroglossia

Question 20

Most common cause of hypothyroidism in children?

Answer 20

autoimmune thyroiditis

Question 21

Zones of the adrenal cortex

Answer 21

Zona glomerulosa = mineralocorticoids
Zona fasciculata = glucocorticoids
Zona reticularis = androgens

Question 22

short term stress response

Answer 22

adrenal medulla release catecholamines (fight or flight response)

Question 23

long term stress response

Answer 23

CRH to anterior pituitary, secretes ACTH, acts on adrenal cortex to release glucocorticoids and mineralocorticoids

Question 24

Actions of glucocorticoids

Answer 24

1. proteins and fats converted to glucose or broken down for energy
2. increased blood glucose
3. suppression of immune system

Question 25

Actions of angiotensin II

Answer 25

stimulates aldosterone secretion from the adrenal cortex

Question 26

Ambiguous genitalia of a newborn

Answer 26

commonly genetically female; commonly congenital adrenal hyperplasia causing virilization of the genitalia

Question 27

Most common deficient enzyme in congenital adrenal hyperplasia

Answer 27

21-hydroxylase; results in deficiency in aldosterone and cortisol and increased production of androgens

Question 28

Labs results of 21-hydroxylase deficiency

Answer 28

low aldosterone
low cortisol
high androgens

Question 29

Treatment for infant in crisis due to congenital adrenal hyperplasia

Answer 29

IV or IM hydrocortisone
IV fluids/glucose
Management of hyperkalemia

Question 30

Addison’s disease

Answer 30

primary adrenal insufficiency; adrenal dysfunction/destruction due to autoimmune reaction; fatigue, reduced stamina, skin hyperpigmentation

Question 31

Lab finding in patient with Addison’s disease?

Answer 31

low 8AM plasma cortisol accompanied by simultaneous significant elevation of plasma ACTH

Question 32

Lab findings of patient suffering from acute adrenal crisis

Answer 32

metabolic acidosis
Others: low serum cortisol, low blood sugar, low sodium, high potassium

Question 33

main treatment for acute adrenal crisis

Answer 33

Hydrocortisone
fluids/glucose
fludrocortisone (after hydrocortisone)
if needed, treat hyperkalemia

Question 34

Abnormal lab result indicative of Cushing’s syndrome

Answer 34

elevated midnight cortisol levels

Question 35

the best screening for Cushing’s syndrome

Answer 35

failed dexamethasone stimulation test; not suppressed by low levels, suppressed by high doses (ectopic Cushing’s is not suppressed by low or high levels)

Question 36

carcinoma associated with ectopic ACTH syndrome

Answer 36

small cell carcinoma of the lung

Question 37

List major signs and symptoms of primary aldosteronism?

Answer 37

low renin HTN
hypokalemia
metabolic acidosis

Question 38

Conn syndrome

Answer 38

adrenal adenoma; causes primary aldosteronism

Question 39

Pheochromocytomas

Answer 39

adrenal medulla tumor; secretes catecholamines

Question 40

Paraganglioma

Answer 40

tumor outside of the adrenal medulla that secretes catecholamines

Question 41

most sensitive diagnostic test for pheochromocytomas and paragangliomas

Answer 41

plasma fractional free metanephrines

Question 42

von Hippel-Lindau disease type 2

Answer 42

AD; patients will have:
retinal capillary hemangiomas (blood vessel tumors)
hemangioblastomas of the CNS
increased risk for renal cysts that transform to renal cell carcinoma

Question 43

Why is it important to treat with an alpha block PRIOR to an beta-blocker in a patient wit pheochromocytoma?

Answer 43

unopposed alpha-receptor stimulation can lead to further elevation of BP