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Flashcards in Adrenals--Newman Deck (35)
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1

how do you confirm the dx of CAH?

17-hydroxyprogesterone increased!

2

CAH- tx

-hydrocortisone
-fluids
-dec K levels

3

Primary adrenal insufficiency- results in?

-dec mineralocorticoids, glucocorticoids, and adrenal androgens, despite an inc conc of ACTH!

4

primary adrenal insufficiency (Addison dz)- caused by

-adrenal destruction
-adrenal dysgenesis (congenital)
-impaired steroidogenesis

5

primary adrenal insufficiency- adrenal destruction- caused by

-autoimmune- most common cause in the US!! (PGA syndrome)
-X-linked recessive disorder- may mimic MS!!!
-infection- TB is most common!!!
-Waterhouse-Friderichsen syndrome!! (hemorrhage)

6

primary adrenal insufficiency- adrenal dysgenesis- caused by

-familial glucocorticoid def (autosomal recessive)- mutations in adrenal ACTH R's

7

primary adrenal insufficiency- impaired steroidogenesis- cuased by

CAH (congenital adrenal hyperplasia)
-def of aldosterone (salt wasting)
-def of cortisol
-excessive androgens (virilization of females)

8

CAH- 21-hydroxylase def

-Mineralocorticoids- DEC
-Androgens (T)- inc

9

CAH- 17-alpha hydroxylase def

-Mineralocorticoids- inc
-androgens- DEC

10

CAH- 11-B hydroxylase def

-Mineralocorticoids- inc
-androgens- inc

11

Primary adrenal insufficiency- signs, sx's

-fatigue, weakness, anorexia, weight loss
-skin pigmentation!!- inc ACTH and MSH
-female infants with 21-hydroxylase def- virilized genitalia

12

Primary adrenal insufficiency- lab

-low Na
-high K
-fasting hypoglycemia
-hypercalcemia
-low 8am plasma cortisol accompanied by simultaneous significant elevation of plasma ACTH

13

primary adrenal insufficiency- confirmatory test

Cosyntropin (synthetic ACTH) stim test
-if cortisol levels dont respond to cosyntropin admin- confirms primary adrenal insuff!!!

14

21-hydroxylase-def- what levels are required for dx?

-elevated 17-OH progesterone

15

Primary adrenal insufficiency- tx

-glucocorticoid replacement tx
-mineralocorticoid replacement tx

16

Acute Adrenal Crisis- tx

-hydrocortisone
-fluids
*make sure to draw 17-OH progesterone level in infants where 21-hydroxylase def is a possibility

17

Secondary adrenal insufficiency

def secretion of ACTH from pit
-low serum ACTH and low serum cortisol!!!!

18

Cushing syndrome- categories

ACTH-dep:
-cushing dz (ACTH-secreting pit adenoma)- 40%
-ectopic ACTH- small cell carcinoma of lung
ACTH-indep:
-exogenous corticosteroid use (most common!!)
-adrenocortical tumor

19

Cushing syndrome- screening test

Dexamethasone suppression test

20

Primary hypercortisolism- tests

-ACTH low
-cortisol- not suppressed (dexamethasone suppression test)

21

Ectopic ACTH syndrome- tests

-ACTH elevated
-cortisol- not suppressed (dexamethasone suppression test)

22

Cushing dz- tests

-ACTH normal/elevated
-cortisol- suppressed by high doses (dexamethasone suppression test)

23

Adrenal incidentalomas- are what? requires what??

adrenal nodules discovered incidentally on abd CT or MRI
**require testing for pheochromocytoma with plasma fractionated free metanephrines!!!

24

Primary hyperaldosteronism

inappropriately high aldosterone secretion that doesnt suppress adequately with Na loading

25

Primary aldosteronism- caused by

-Conn syndrome- aldosterone-producing adrenal adenoma- 40% mutation in gene coding for K channel
-unilateral or b/l adrenal hyperplasia- most common cause!!
-CV complications- more freq in hyperaldosteronism than in idiopathic HTN

26

primary aldosteronism- signs, sx's

-HTN
-hypokalemia

27

test for primary aldosteronism in all HTN pts with what?

-hypokalemia
-tx-resistant HTN (despite 3 drugs)
-severe HTN (>160/100)
-early onset HTN
-low-renin HTN
-HTN with an adrenal mass
-HTN with fam hx of early onset HTN or CVA < 40 yo
-1st degree relative with aldosteronism

28

all pts with primary aldosteronism require what imaging?

-thin-section CT scan of adrenals to screen for adrenal carcinoma (although rare)

29

pheochromocytomas and paragangliomas- are what

tumors of symp NS

30

pheochromocytoma- familial assoc?

VHL disease type 2 (autosomal dominant)
-hemangiomas
-other tumors