Term
| effects of cortisol on metabolism, bone, skin, renal, and immune systems |
|
Definition
| increases GNG, protein catabolism, and lipolysis; inhibits bone formation, breaks down collagen; can bind to mineralcorticoid receptors; inhibits immune system |
|
|
Term
| clinical presentation of Cushing's Syndrome: metabolic, bone, skin, renal, and immune |
|
Definition
| muscle wasting, obesity, moon facies, hirsutism, osteoporosis/fractures, thin skin, striae, hypertension, multiple infections |
|
|
Term
| how do you establish hypercortisolism? |
|
Definition
| 24h urine free cortisol test OR overnight dexamethasone suppression test |
|
|
Term
| what can cause a false positive on UFC test? (4) |
|
Definition
| alcohol, stress, carbamezepine, high urine volume |
|
|
Term
| alcohol, stress, renal failure, and obesity may give you a _____ on the overnight dexamethasone suppression test |
|
Definition
|
|
Term
| you have confirmed that a pt has hypercortisolism. what is your next step? |
|
Definition
| plasma ACTH. high ACTH -> pituitary disease or ectopic tumor. low ACTH -> primary adrenal disease |
|
|
Term
| how do you confirm a pituitary ACTH secreting tumor? |
|
Definition
| MRI or IPSS/CSS. A lesion >5mm -> CD. A central-to-peripheral gradient > 2:1 -> CD. |
|
|
Term
| how do you confirm an adrenal tumor? |
|
Definition
| CT scan or IPSS:periphery < 3:1 |
|
|
Term
| how do you confirm an ectopic ACTH tumor? |
|
Definition
| IPSS. IPSS:periphery <3:1 -> ectopic ACTH tumor |
|
|
Term
|
Definition
| surgery is best. medical tx includes metyrapone, ketoconazole, mifepristone, mitotane, and aminoglutethimide |
|
|
Term
| what drug inhibits 11beta-hydroxylase |
|
Definition
|
|
Term
| what drug inihibits 11beta-hydroxylase and cholesterol side chain cleavage |
|
Definition
|
|
Term
| causes of primary adrenal insufficiency (5) |
|
Definition
| autoimmune (Addison's); TB, infections, tumor, hemorrhage |
|
|
Term
| most common cause of secondary adrenal insufficiency |
|
Definition
| space-occupying lesion in pituitary (abscess, aneurysm, tumor) |
|
|
Term
| adrenal hormone levels in primary adrenal insufficiency vs. secondary adrenal insufficiency |
|
Definition
primary: all decreased
secondary: normal aldosterone; decreased cortisol and sex hormones |
|
|
Term
| hyponatremia, hyperkalemia, hypotension, hypoglycemia, hyperpigmentation; dx? |
|
Definition
| primary adrenal insufficiency |
|
|
Term
| hyponatremia, no hyperkalemia, hypoglycemia, hypopigmentation; dx? |
|
Definition
| secondary adrenal insufficiency |
|
|
Term
| how do you establish adrenal insufficiency? |
|
Definition
| ACTH stimulation test. Measure cortisol. Neither primary nor secondary should respond. |
|
|
Term
| how do you distinguish primary from secondary adrenal insufficiency, diagnostically? |
|
Definition
Measure PRA, aldosterone, and ACTH.
Primary: high PRA, low aldosterone, high ACTH
Secondary: normal PRA, normal aldosterone, low ACTH |
|
|
Term
| how do you dx acute adrenal insufficiency? |
|
Definition
insulin challenge or overnight metyrapone.
insulin: hypoglycemia -> stress -> cortisol secretion. measure cortisol after 1h. cortisol>20ug/dl is normal.
overnight metyrapone: metyrapone inhibits 11-beta hydroxlyase. Measure DOC. DOC>7ug/dl is normal. |
|
|
Term
| tx for primary vs secondary adrenal insufficiency |
|
Definition
primary: glucocorticoid + mineralcorticoid replacement
secondary: glucocorticoid replacement |
|
|
Term
| what enzymes are commonly mutated in CAH? |
|
Definition
| 21-hydroxylase, 17a-hydroxylase, and 11b-hydroxylase |
|
|
Term
| what steroids are produced by 21-hydroxylase? 11b-hydroxylase? 17a-hydroxylase? |
|
Definition
21-OH: aldosterone, cortisol
11b-OH: aldosterone, cortisol
17a-OH: cortisol, sex hormones |
|
|
Term
| precocious puberty in males, virilizing in females, +/- salt wasting, hyperkalemia if severe; dx? |
|
Definition
|
|
Term
| asymptomatic male, hirsutism in females at puberty; no salt wasting; dx? |
|
Definition
|
|
Term
| virilized females at puberty, precocious puberty in males, no salt wasting |
|
Definition
| 21-OH or 11b-OH deficiency |
|
|
Term
| what prevents salt-wasting in 11b-hydroxylase deficiency? |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| ambiguous sex at birth in males; normal at birth for females; neither sexually mature; dx? |
|
Definition
|
|
Term
| HTN in ambiguous male; dx? |
|
Definition
|
|
Term
| HTN in a virilized female; dx? |
|
Definition
|
|
Term
| hypotension in a male with precocious puberty; dx? |
|
Definition
|
|
Term
| HTN, no edema, decreased PRA, tetany, muscle spasms; dx? |
|
Definition
|
|
Term
| HTN, no edema, increased PRA, tetany, muscle spasms; dx? |
|
Definition
|
|
Term
| what are the 2 causes of primary aldosteronism? |
|
Definition
| bilateral adrenal hyperplasia or unilateral adrenal tumor |
|
|
Term
| how do you screen for primary aldosteronism? |
|
Definition
measure aldosterone:renin ratio
if AR ratio>15 and serum aldosterone>15ng/ml, then workup for primary aldosteronism |
|
|
Term
| most common cause of primary aldosteronism is... |
|
Definition
| bilateral adrenal hyperplasia |
|
|
Term
| who is recommended for screening? (4) |
|
Definition
| pts with refractory HTN, young pts with HTN, family hx, adrenal incidentaloma |
|
|
Term
| how do you confirm primary aldosteronism? (3) |
|
Definition
adrenal CT scan, salt load and measure urine aldosterone, or volume expansion test
volume expansion: give 2L of saline IV over 4h; this should cause hyponatremia, suppress aldosterone secretion, and increase salt excretion; aldosterone>10ng/dl -> primary aldosteronism |
|
|
Term
| tx for bilateral adrenal hyperplasia vs. adenoma? |
|
Definition
hyperplasia: eplerenone
adenoma: surgery |
|
|
Term
| pathology of secondary aldosteronism |
|
Definition
| renal artery stenosis reduces blood flow to glomeruli -> increases renin secretion -> increases aldosterone production |
|
|
Term
| HTN with decreased aldosterone and decreased PRA; dx? |
|
Definition
|
|
Term
| 11b-hydroxysteroid dehydrogenase deficiency causes... |
|
Definition
|
|
Term
|
Definition
| excess cortisol production due to 11b-hydroxysteroid dehydrogenase deficiency; cortisol can bind mineralcorticoid receptors |
|
|
Term
|
Definition
|
|
