Clinical Adrenal Cases

Question 0

Triad associated with primary adrenal insufficiency.

Answer 0

Weakness, fatigue, anorexia

Question 1

Most common cause of primary adrenal insufficiency.

Answer 1

Autoimmune Adrenalitis

Question 2

What basal level (early morning) and stress level cortisol plasma measurements are strong indicators of adrenal insufficiency?

Answer 2

Basal: less than 3ug/dL
Stress: less than 5ug/dL

Question 3

Name and describe the best test to differentiate primary Vs secondary adrenal insufficiency.

Answer 3

High Dose ACTH test.

1. Measure baseline plasma ACTH and cortisol at 8AM
2. Inject ‘Cosyntropin’, an ACTH analog, 250mg
3. Measure serum cortisol 30min after injection, then 60 min after.
4. Measure serum cortisol and ACTH again.

ACTH greater than 100pg/ml and low cortisol response (less than 18pg/ml) means primary AI

Normal Or low ACTH with elevated cortisol would lean more towards secondary or tertiary AI

Question 4

How is a patient with adrenal insufficiency handled if they become unstable while finding the source of the insufficiency?

Answer 4

Treat immediately with glucocorticoids. Hydrocortisone is standard, however if you are performing the ACTH stimulation test, Dexamethasone can be used and will not alter the test results.

Question 5

Treatment for primary adrenal insufficiency.

Answer 5

Life-long glucocorticoid

• 2/3 of total daily amount in morning
• 1/3 of total daily amount in the evening

and mineralocorticoid therapy

Question 6

Most common cause of secondary adrenal insufficiency.

Answer 6

A patient taking glucocorticoids for some reason for at least 3 weeks, then suddenly stops. The medication suppresses the hyp-pit axis and prevents CRH and ACTH release. Even after stopping the medication, this suppression can continue for months.

Question 7

What are some clinical differences between secondary AI and primary AI?

Answer 7

Hyper pigmentation only in primary
Hypo aldosteronism only in primary
Hypoglycemia more common in secondary

Question 8

Tx for secondary AI caused by cessation of exogenous glucocorticoid therapy.

Answer 8

Immediately restart the therapy, then slowly taper them off the medication

Question 9

Who is at risk of Adrenal Crisis

Answer 9

Anybody with damage to the adrenal cortex causing an acute cessation of aldosterone secretion. This can be poorly managed primary AI, someone with primary AI that stops taking meds abruptly, secondary AI with abrupt cessation of meds, bilateral adrenal infarction.

The problem is, the lack of aldosterone drops the blood pressure substantially and rapidly leading to shock and eventually death.

Question 10

What is the emergent treatment for adrenal crisis?

Answer 10

Immediately gain IV access to check cortisol and ACTH levels

Begin IV saline and glucocorticoids

(5S’s: salt, sugar, steroids, support, search for etiology)

Question 11

Most common cause of Cushing Syndrome.

Answer 11

Exogenous glucocorticoid use

Question 12

Name the 3 screening tools used to Dx Cushing Syndrome

Answer 12

1. 24hr Urine Free Cortisol Test (most common)
2. 1mg Dexamethasone Suppression Test
3. Midnight Salivary Cortisol Test

Question 13

How is the 24 urine Free cortisol test interpreted?

Answer 13

Check the urine for creatinine levels.

Values 3-4 times normal along with symptoms is diagnostic for Cushing Syndrome

Question 14

How is the 1mg Dexamethasone Suppression test interpreted?

Answer 14

Administer dexamethasone at night, measure cortisol levels in the morning.

Cortisol less than 1.8ug/dL is normal, greater than 1.8 indicates Cushing Syndrome

Question 15

What ACTH levels are used to determine ACTH independent or dependent Cushing Syndrome?

Answer 15

Dependent: greater than 20ug/dL
Independent: less than 10ug/dL

Question 16

Tx for Cushing Syndrome due to Adrenal Adenoma.

Answer 16

Surgical Unilateral Adrenalectomy

Question 17

Triad in a patient with pheochromocytoma.

Answer 17

Sudden Severe Headaches
Diaphoresis
Palpitations

Question 18

Main test to diagnose pheochromocytoma.

Answer 18

24 hr urine collection for free catecholamines, metanephrines, normetanephrines

-metanephrines and normetanephrines are just Epi and NE metabolites after interaction with MOA and COMT. In biochem we just learned to look for Vanillylmandelic acid (VMA) which is the last common product in the degredation pathway of catecholamines.

Question 19

How does a pheochromocytoma appear on MRI?

Answer 19

Light Bulb Sign

Question 20

How is Pheochromocytoma treated?

Answer 20

Step 1: alpha blockade the patient with phenoxybenzamine 7-10 days before surgery.

Step 2: surgical removal of affected adrenal gland

Question 21

Most common type of primary hyperaldosteronism (Conn Syndrome).

Answer 21

Solitary Aldosterone Producing Adenoma

Question 22

Second most common cause of Conn Syndrome.

Answer 22

Bilateral Adrenal Glomerular Hyperplasia (idiopathic hyperaldosteronism)

Question 23

What are the 3 main groups of people that should be screened for primary hyperaldosteronism?

Answer 23

1. Patients with HTN and hypokalemia (spontaneous or diuretic-induced)
2. Patients with severe HTN (160/100)
3. Patients with drug-resistant HTN (on 3+ bp meds that don’t respond)

Question 24

Describe screening for primary hyperaldosteronism.

Answer 24

Measure Plasma Renin Activity (PRA)
Measure Plasma Aldosterone Conc. (PAC)
Calculate ratio of PAC/PRA

If PAC measures greater than 15ng/dL
AND
PAC/PRA is greater than 20
diagnostic for Primary Hyperaldosteronism

Question 25

Once Primary Hyperaldosteronism is diagnosed. What are the next 3 steps for treatment?

Answer 25

1. Check CT to plan surgery
2. Adrenal Vein sampling to confirm unilateral disease
3. If unilateral: perform a unilateral adrenalectomy
   If bilateral: use mineralocorticoid antagonist medication

Question 26

What medications are used to treat bilateral hyperaldosteronism?

Answer 26

Spironolactone: more side effects but cheaper

Eplerenone: less side effects due to high specificity for mineralocorticoid receptors.