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Endocrine 2/3
Adrenals
Question | Answer |
---|---|
What is the difference in androgen production between iatrogenic Cushing's syndrome and Cushing's disease? | Iatrogenic has no excess androgens b/c the exogenous steiod suppresss androgen production by the adrenals. In Cushing's disease, androgen excess is common. |
Which type of lung cancer can cause ectopic ACTH production? | Small cell (2/3 of cases). (Also bronchial carcinoids and thymomas can cause ectopic ACTH production) |
What effect does cortisol have on immunity? | Impairs it (inhibits PMNs and T cells) |
Which is more likely to have hyperpigmentation: Cushing's syndrome or Cushing's disease? | Cushing's disease (due to elevated ACTH levels) |
Which serum cortisol level on an overnight low-dose DXM suppression test effectively rules out Cushing's syndrome? | Serum cortisol <5 (serum cortisol >5 or 10= Cushing's syndrome use high DXM suppression to determine cause) |
What's the diagnosis?: high dose DXM test with >50% reduction in Cortisol levels | Cushing's disease |
What's the diagnosis?: high dose DXM test with no cortisol suppression and high plasma ACTH | ectopic ACTH-producing tumor |
What's the diagnosis?: CRH stimulation test with increase in ACTH/cortisol levels | Cushing's disease |
What's the diagnosis?: CRH stimulation test with no increase in ACTH/cortisol levels | Ectopic ACTH secretion or adrenal tumor |
Which syndrome is associated with pheochromocytoma? | MEN II |
What is the MC site of extra-adrenal pheos (10% of pheos)? | organ of Zuckerkandl, located at aortic bifurcation |
If you suspect an adrenal tumor or hyperplasia causing Cushing's syndrome, what test/lab should be ordered? | Adrenal imaging |
What studies should be ordered to confirm a diagnosis of ectopic ACTH-producing tumor? | Chest CT, Abdominal CT, and Octreotide scan |
What is the nature of BP in pheos? | Persistently high BP with episodic severe HTN |
Name 3 lab findings in pheo. | Hyperglycemia, hyperlipidemia, and hypokalemia |
Pt presents with headache, profuse sweating, palpitations, and fear/sense of impending doom. Likely dx? | Pheo until proven otherwise |
Best urinary indicator of pheo? | Metanephrine |
How do you know a pheo must be adrenal? | Elevated urine/serum epinephrine (non-adrenals cannot methylate norepi--> epi) |
Tx of choice for pheo? | Surgical resection with early ligation of venous draininage (ligation lowers possibility of catecholamine release/crisis); also medical with alpha blockers (phenoxybenzamine) |
3 MEN I (Werner's) | 1. Parathyroid hyperplasia 2. Pancreatic islet cell tumors (Z-E syndrome) 3. Pituitary tumors |
3 MEN IIa (Sipple's) | 1. Medullary thyroid CA 2. Pheo 3. Hyperparathyroidism |
4 MEN IIb | 1. Mucosal neuromas 2. Medullary thyroid CA 3. Marfanoid body habitus 4. Pheo |
Hypertensive pt with hypokalemia who is not on diuretic and has no peripheral edema? | Suspect hyperaldosteronism |
Aldosterone-producing adrenal adenoma | Conn's (hyperaldosteronism) |
What causes nocturnal polyuria in primary hyperadolsteronism? | Hypokalemia |
Plasma aldosterone: renin level ____ warrants investigation for primary hyperaldosteronism | >30 |
What result of a saline infusion rules out primary hyperaldosteronism? | <8.5ng/dL |
Tx of choice for adrenal adenoma causing hyperaldosteronism? | Surgerical resection (curative) |
Tx of choice for bilateral adrenal hyperplasia causing hyperaldosteronism? | Spironolactone (inhibits action of aldost). Surgery is NOT indicated! |
Primary adrenal insufficiency= | Addison's disease |
MCC of primary adrenal insufficiency worldwide? In Western world? | TB worldwide; autoimmune Western |
MCC secondary adrenal insufficiency? | Pts on long-term steroid therapy (pts with serious illness or trauma unable to release appropriate amt of cortisol); also MCC adrenal insufficiency overall |
Addison's effect on: glucose, BP, pigmentation | hypOglycemia, hypOtension, hyperpigmentation (seen in primary ONLY) |
Why is hyperpigmentation not seen in secondary adrenal insufficiency? | B/c ACTH levels are low in secondary AI (either pit is not releasing ACTH b/c it's low-functioning or exogenous steroids are suppressing release of ACTH) |
Addison's effect on: aldosterone levels, sodium levels, volume status, and potassium levels | Low aldosterone, low sodium, hypovolemia, hyperkalemia |
How can ACTH test distinguish btwn primary and secondary adrenal insufficiency? | Primary: cortisol will not increase sufficiently with an ACTH infusion Secondary: after 4-5 days of treatment, adrenals "wake up" and begin responding normally to ACTH stimulation (will be sluggish initially) |
What test should be ordered if secondary or tertiary adrenal insufficiency is diagnosed? | MRI of brain (pit/hypothalamus) |
Tx for primary adrenal insufficiency? | Daily oral glucocorticoid (hydrocortisone or prednisone) + daily fludrocortisone (mineralcorticoid) |
Tx for secondary adrenal insufficiency? | Daily oral glucocorticoid (prednisone or hydrocortisone); daily mineralcorticoid (fludrocortisone) in unnecessary |
MCC of congenital adrenal hyperplasia(CAH)? | 21-hydroxylase deficiency (11-hydroxylase is 2nd MCC) |
What causes the hyperplasia in congenital adrenal hyperplasia (CAH)? | Decreased cortisol and aldosterone production--> increased ACTH--> hyperplasia of glands |
What causes the virilization seen in CAH? | precursors to cortisol and aldosterone build up--> shunted toward synthesis of androgens (DHEA, testosterone) --> virilization |
Females infants born with CAH? Males? | Ambiguous genitalia, but normal female ovaries and uterus. Male infants have no genital abnormalities. |
Emesis, dehydration, hypOtension, and shock in first 2-4 weeks of life? | Salt-wasting form of CAH |
CAH effect on: sodium, potassium, glucose | hypOnatremia,hyperkalemia, hypOglycemia (due to lack of cortisol) |
How is CAH diagnosed? | High levels of 17-hydroxyprogesterone in serum |
Tx for CAH? | Medical: use cortisol and mineralocorticoid to shut off excess ACTH secretion Surgical: correction of genital abnormalities recommended |