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Flashcards in ped adrenal disorders Deck (31)
1

most common form of adrenal steroid synthesis

deficiency of 21 hydroxylase

2

most cases of 21 hydroxylase defiency are salt-losing or not?

salt losing

3

hyponatremia, hyperkalemi, hypoglycemia, FTT

classic 21 hydroxylase deficiency

4

non-classic CAH (21 hydroxylase deficiecy)

non-salt losing

5

later presentation, menstrual irregularity, early pubarche, sexual precocity in females

non-classic CAH (21 hydroxylase deficiecy)

6

HTN, virilization, non-salt losing

11-beta hydroxylase deficiency

7

non-salt losing, HTN, hypokalemia, ambiuous genitalia in males, no secondary sexual development in females

17 hydroxylase deficiency

8

what to check in case of 21 hydroxylase defect?

17-hydroxyprogesterone will be elevated

9

in 21 hydroxylase deficiency, ACTH will be___, renin will be____

elevated; elevated

10

what is the gold standard for dx of 21 hydroxylase deficiency

consyntopin stimulation test

11

11 beta hydroxylase defect will have elevated (2)

1. 11-deoxycorticosterone
2. 11-deoxycortisol

12

17 hydroxylase defect will have elevated____, decr___

aldosterone levels , potassium

13

develops gradually with sx starting b/w age 10-12

addisons disease

14

most common cause of addisons

autoimmune destruction of adrenal gland

15

which autoimmune polyglandular syndrome? hypoparathyroidism, chronic mucocutaneous candidiasis, adrenal insufficiency

APS 1

16

which autoimmune polyglandular syndrome? adrenal insufficiency, hashimotos thyroiditis, type 1 DM

APS 2 (schmidt syndrome)

17

is APS 1 or 2 more common?

APS 2

18

how to confirm low cortisol secretion

morning cortisol levels

19

muscular weakness, dehydration, weight loss, hypotension hyponatreia, hypoglycemia, hyperpigmentation

adrenal insufficiency

20

in ______there will be no response to cosyntropin test b/c ACTH is already elevated

primary adrenal insufficiency

21

chronic tx for primary adrenal insufficiency

hydrocortisone and fludrocortisone

22

if PAI patient under stress, incr hydrocortison dose to ____

3-4x normal

23

most common cause of cushing syndrome in infants

adrenal tumor

24

most common cause of cushing syndrome in children

ACTH-secreting pituitary adenoma

25

1st step in dx of cushings (2 options)

1. 24 hr urinary cortisol
2. low dose dexamethasone

26

test to determine reason for high ACTH

high dose dexamethasone test

27

pheochromocytoma usu presents during age____

6-14

28

tumor of the chromaffin cells, MEN 2A and 2B syndromes

pheochromocytoma

29

von-hippel lindau syndrome assc'd with?

pheochromocytoma

30

unlike adults, children have______HTN

sustained

31

tx pheochromocytoma with ____, then _____

phenocybenzamine, then beta blockers