- hydrocortisone - fluids - dec K levels

Adrenals--Newman Flashcards by Sammy Adhikari

how do you confirm the dx of CAH?

17-hydroxyprogesterone increased!

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CAH- tx

hydrocortisone
fluids
dec K levels

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Primary adrenal insufficiency- results in?

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

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primary adrenal insufficiency (Addison dz)- caused by

adrenal destruction
adrenal dysgenesis (congenital)
impaired steroidogenesis

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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)

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primary adrenal insufficiency- adrenal dysgenesis- caused by

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

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primary adrenal insufficiency- impaired steroidogenesis- cuased by

CAH (congenital adrenal hyperplasia)

def of aldosterone (salt wasting)
def of cortisol
excessive androgens (virilization of females)

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CAH- 21-hydroxylase def

Mineralocorticoids- DEC

- Androgens (T)- inc

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CAH- 17-alpha hydroxylase def

Mineralocorticoids- inc

- androgens- DEC

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CAH- 11-B hydroxylase def

Mineralocorticoids- inc

- androgens- inc

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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

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Primary adrenal insufficiency- lab

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

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primary adrenal insufficiency- confirmatory test

Cosyntropin (synthetic ACTH) stim test

-if cortisol levels dont respond to cosyntropin admin- confirms primary adrenal insuff!!!

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

-elevated 17-OH progesterone

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Primary adrenal insufficiency- tx

glucocorticoid replacement tx

- mineralocorticoid replacement tx

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Acute Adrenal Crisis- tx

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

Secondary adrenal insufficiency

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

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

Cushing syndrome- screening test

Dexamethasone suppression test

Primary hypercortisolism- tests

ACTH low

- cortisol- not suppressed (dexamethasone suppression test)

Ectopic ACTH syndrome- tests

ACTH elevated

- cortisol- not suppressed (dexamethasone suppression test)

Cushing dz- tests

ACTH normal/elevated

- cortisol- suppressed by high doses (dexamethasone suppression test)

Adrenal incidentalomas- are what? requires what??

adrenal nodules discovered incidentally on abd CT or MRI

**require testing for pheochromocytoma with plasma fractionated free metanephrines!!!

Primary hyperaldosteronism

inappropriately high aldosterone secretion that doesnt suppress adequately with Na loading

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

primary aldosteronism- signs, sx's

- HTN | - hypokalemia

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

all pts with primary aldosteronism require what imaging?

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

pheochromocytomas and paragangliomas- are what

tumors of symp NS

pheochromocytoma- familial assoc?

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

pheochromocytoma- most sensitive test

-plasma fractionated free metanephrines!!

surgery/resection of pheochromocytoma- can cause what?

pheochromocytomatosis | -abdomen can be "seeded" during surgery

pheochromocytoma- medical tx prior to resection | alpha-blockers

-phenoxybenzamine- long acting nonselective alpha-blocker!!

detecting metastatic pheochromocytoma

-surgical histopathology for pheochromocytoma and paraganglioma CANNOT reliably determine whether tumor is malignant

Multiple Endocrine Neoplasia (MEN)

- autosomal dominant - predisposition to the development of tumors in 2 or more endocrine glands - MEN 2 and 3 are assoc with pheochromocytomas