Adrenals--Newman Flashcards

1
Q

how do you confirm the dx of CAH?

A

17-hydroxyprogesterone increased!

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2
Q

CAH- tx

A
  • hydrocortisone
  • fluids
  • dec K levels
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3
Q

Primary adrenal insufficiency- results in?

A

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

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4
Q

primary adrenal insufficiency (Addison dz)- caused by

A
  • adrenal destruction
  • adrenal dysgenesis (congenital)
  • impaired steroidogenesis
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5
Q

primary adrenal insufficiency- adrenal destruction- caused by

A
  • 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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6
Q

primary adrenal insufficiency- adrenal dysgenesis- caused by

A

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

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7
Q

primary adrenal insufficiency- impaired steroidogenesis- cuased by

A

CAH (congenital adrenal hyperplasia)

  • def of aldosterone (salt wasting)
  • def of cortisol
  • excessive androgens (virilization of females)
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8
Q

CAH- 21-hydroxylase def

A
  • Mineralocorticoids- DEC

- Androgens (T)- inc

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9
Q

CAH- 17-alpha hydroxylase def

A
  • Mineralocorticoids- inc

- androgens- DEC

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10
Q

CAH- 11-B hydroxylase def

A
  • Mineralocorticoids- inc

- androgens- inc

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11
Q

Primary adrenal insufficiency- signs, sx’s

A
  • fatigue, weakness, anorexia, weight loss
  • skin pigmentation!!- inc ACTH and MSH
  • female infants with 21-hydroxylase def- virilized genitalia
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12
Q

Primary adrenal insufficiency- lab

A
  • low Na
  • high K
  • fasting hypoglycemia
  • hypercalcemia
  • low 8am plasma cortisol accompanied by simultaneous significant elevation of plasma ACTH
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13
Q

primary adrenal insufficiency- confirmatory test

A

Cosyntropin (synthetic ACTH) stim test

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

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14
Q

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

A

-elevated 17-OH progesterone

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15
Q

Primary adrenal insufficiency- tx

A
  • glucocorticoid replacement tx

- mineralocorticoid replacement tx

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16
Q

Acute Adrenal Crisis- tx

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

Secondary adrenal insufficiency

A
def secretion of ACTH from pit
-low serum ACTH and low serum cortisol!!!!
18
Q

Cushing syndrome- categories

A

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
Q

Cushing syndrome- screening test

A

Dexamethasone suppression test

20
Q

Primary hypercortisolism- tests

A
  • ACTH low

- cortisol- not suppressed (dexamethasone suppression test)

21
Q

Ectopic ACTH syndrome- tests

A
  • ACTH elevated

- cortisol- not suppressed (dexamethasone suppression test)

22
Q

Cushing dz- tests

A
  • ACTH normal/elevated

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

23
Q

Adrenal incidentalomas- are what? requires what??

A

adrenal nodules discovered incidentally on abd CT or MRI

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

24
Q

Primary hyperaldosteronism

A

inappropriately high aldosterone secretion that doesnt suppress adequately with Na loading

25
Q

Primary aldosteronism- caused by

A
  • 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
Q

primary aldosteronism- signs, sx’s

A
  • HTN

- hypokalemia

27
Q

test for primary aldosteronism in all HTN pts with what?

A
  • 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
Q

all pts with primary aldosteronism require what imaging?

A

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

29
Q

pheochromocytomas and paragangliomas- are what

A

tumors of symp NS

30
Q

pheochromocytoma- familial assoc?

A

VHL disease type 2 (autosomal dominant)

  • hemangiomas
  • other tumors
31
Q

pheochromocytoma- most sensitive test

A

-plasma fractionated free metanephrines!!

32
Q

surgery/resection of pheochromocytoma- can cause what?

A

pheochromocytomatosis

-abdomen can be “seeded” during surgery

33
Q

pheochromocytoma- medical tx prior to resection

alpha-blockers

A

-phenoxybenzamine- long acting nonselective alpha-blocker!!

34
Q

detecting metastatic pheochromocytoma

A

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

35
Q

Multiple Endocrine Neoplasia (MEN)

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