Large Group 2

Question 1

What is the difference between Cushing’s Disease and Cushing’s Syndrome?

Answer 1

Disease=ACTH secreting pituitary adenoma

syndrome=all causes of excess cortisol (exogenous ACTH tumor, adrenal tumor, excessive GC treatment, ectopic)

Question 2

What is often the cause of ectopic ACTH release?

Answer 2

small cell lung cancer

Question 3

What skin issues are seen in Cushing’s disease?

Answer 3

broad cutaneous vilatious striae

easy bruising

red face

Question 4

What are the 2 classifications of endogenous Cushing’s Syndrome?

Answer 4

ACTH independent (adrenally producing excess cortisol regardless of ACTH level)

ACTH dependent (includes pituitary and ectopic issues producing elevated ACTH causing the excess cortisol)

Question 5

What do the AM and PM cortisol test and the midnight salivary cortisol test test for?

Answer 5

loss of diurnal variation in cortisol release seen in Cushing’s

AM and PM: AM should be > PM in normal person

midnight salivary: cortisol is expected to be lower at night in normal people.

Question 6

How does the low dose Dexamethasone test work? What do the results tell you about the cause of the problem?

Answer 6

1mg Dexamethasone given at midnight (it is a long acting deus with no MC activity)

normal=plasma cortisol < 5 mg/dL the next morning

elevated cortisol=pituitary or adrenal problem (look at the starting ACTH levels to determine which)

Question 7

What is the 24 hour free cortisol test?

Answer 7

have people collect their urine for 24 hours

> 140 nmol/d is suggestive of Cushing’s

Question 8

Which will produce more ACTH, a pituitary tumor or an ectopic tumor?

Answer 8

ectopic

Question 9

If an adrenal adenoma is causing Cushing’s, what will the ACTH be? What about a pituitary or ectopic tumor?

Answer 9

adrenal: low ACTH

pituitary or ectopic: high ACTH

Question 10

How will the different tumors respond to HIGH dose (8mg) Dexamethasone? What does this help determine?

Answer 10

adrenal: cortisol will still be high

ectopic (lung cancer): cortisol will remain high.

Pituitary microadenoma: decrease in the cortisol production (from the feedback inhibition of ACTH)

Pituitary Macroadenoma: cortisol will be HIGH (not responsive to the high Dex)

helps determine micro vs macro adenoma

Question 11

What is a useful test in determining the difference between a macro adenoma and an ectopic tumor?

Answer 11

since both will maintain high levels of cortisol with high ACTH, a pituitary MRI would help

if no mass is seen, it is likely ectopic

Question 12

If a pituitary macroadenoma is found, what else should be ordered?

Answer 12

check the different axes for issues

TSH and T4, GH and IGF-1, LH and testosterone

Question 13

If a pt is having a right adrenalectomy for a right adrenal adenoma, what pre-op and post-op treatment should they receive? Why?

Answer 13

pre-op and post-op glucocorticoids (short-acting) with larger doses in the morning and less at night to mimic the diurnal variation

pre-op to keep blood pressure up and post-op to allow the left adrenal time to begin operating again (may have become less functioning because of the previously elevated cortisol levels due to the adenoma)

Question 14

What is an adrenal incidentaloma?

Answer 14

a mass lesion > 1 cm that is found serendipitously by a radiologic exam

Question 15

What do you suspect with HTN, palpitations, sweating and headaches?

Answer 15

pheochromocytoma

Question 16

Which syndrome is not associated with pheochromocytoma?
A. MEN 1
B. MEN 2A
C. MEN 2B 
D. VHL

Answer 16

A. MEN 1 (the 3 P’s that are organs –> pituitary, parathyroid and pancreas)

Question 17

What are the 3 things affected by MEN 2A?

Answer 17

parathyroid

pheochromocytoma

medullary thyroid cancer

Question 18

What are the 3 things affected by MEN 2B?

Answer 18

mucosal neuroma

medullary thyroid cancer

pheochromocytoma

Question 19

When should you consider testing for primary aldosteronism?

Answer 19

HTN and hypkalemia

resistant HTN

adrenal incidentaloma and HTN

HTN onset 160 systolic or >100 diastolic)

whenever considering secondary HTN

Question 20

What is the screening test for primary aldosteronism? What results are necessary for a diagnosis?

Answer 20

Plasma Aldosterone and Renin

PAC (plasma aldosterone concentration) >15 ng/dL or decreased PRA (20 ng/dL

PRA=renin

Question 21

How do you confirm a diagnosis of primary aldosteronism?

Answer 21

try to suppress the aldosterone production

have the pt increase their Na+ to 6g/day x3 days and then collect the urine on the 3rd through the 4th day and verify the amount of sodium in the urine

if the aldosterone secretion is high (>12 mcg/24hr), suspect primary aldosteronism (the elevated Na+ and increase in volume should have caused the aldosterone to decrease)

Question 22

What are the medical treatments available for primary aldosteronism?

Answer 22

mineralocorticoid receptor antagonists

spironolactone–> causes gynecomastia

Question 23

When would you perform surgery in a pt with primary aldosteronism? what option could be considered if the pt was over the age of 40?

Answer 23

surgery in a pt with a mass >1 cm and marked primary aldosteronism under the age of 40

in a pt with the same symptoms over the age of 40, do AVS (adrenal venous sampling) to ensure that there is lateralization prior to the adrenalectomy

Question 24

What will the cortisol level and ACTH be in a pt with primary vs secondary adrenal insufficiency?

Answer 24

primary:
cortisol= low
ACTH=high

secondary
cortisol= low
ACTH =low

Question 25

Will pts with secondary adrenal insufficiency be hyper pigmented?

Answer 25

NO because ACTH will not be elevated

Question 26

What is a possible cause of primary adrenal insufficiency? Secondary?

Answer 26

primary: adrenal hemorrhage
secondary: sudden cessation of exogenous glucocorticoids

Question 27

What can a pt who took GC for a long time and suddenly stopped appear as though they have?

Answer 27

Cushings

Question 28

What test should be done if you suspect adrenal insufficiency?

Answer 28

cosyntropin (synthetic ACTH) stimulation test

if the adrenal gland can be stimulated to make >18 micrograms/dL of cortisol, it rules out adrenal insufficiency

if it cannot make 18 with cosyntropin, and the ACTH at baseline was HIGH, it suggests primary AI

if it cannot make 18 of cortisol and the baseline ACTH was LOW, it suggests secondary AI

Question 29

How does the treatment differ for primary and secondary adrenal insufficiency?

Answer 29

primary: give both glucocorticoids and mineralocorticoids
secondary: only give glucocorticoids (MC are not under the control of ACTH)

Question 30

What should a pt with AI be aware of?

Answer 30

they need to supplement steroids in times of stress