Exam 4: Adrenal Gland

Question 1

What two classes of hormones does the adrenal gland produce?

Answer 1

Steroids and catecholamines

Question 2

Where in the adrenal gland are catecholamines and peptides made?

Answer 2

Medulla

Question 3

Where in the adrenal gland are mineralocorticoids (aldosterone (salt)) made?

Answer 3

The cortex, specifically zona glomerulosa

Question 4

What does aldosterone do?

Answer 4

Regulates salt balance and blood volume

Question 5

What controls the release of aldosterone?

Answer 5

AT II and serum potassium concentrations

Question 6

Where in the adrenal gland are glucocorticoids produced?

Answer 6

Zona fasciculata

Question 7

What does cortisol do?

Answer 7

Increases blood sugar and has many metabolic effects

Question 8

What controls the release of cortisol?

Answer 8

ACTH, CRH

Question 9

Where in the Adrenal Gland are androgens made?

Answer 9

The cortex, specifically the zone reticularis

Question 10

What controls the release of androgens?

Answer 10

ACTH, CRH

Question 11

What controls the release of catecholamines?

Answer 11

Preganglionic sympathetic neurons

Question 12

Cortisol has negative feedback on what two things? This results in decreased production of what?

Answer 12

Cortisol has negative feedback on the hypothalamus, so it does not secrete CRH, and it has negative feedback on the anterior pituitary so it does not secrete ACTH

Question 13

What are the functions of cortisol?

Answer 13

Stimulates gluconeogenesis, decreases glucose uptake by tissues, anti-inflammatory effects, reduces bone formation, increases proteolysis, decreases plasma Ca, and inhibits collagen formation

Question 14

When are cortisol levels the highest?

Answer 14

The morning

Question 15

What results from prolonged exposure to excess glucocorticoids and androgens?

Answer 15

Cushing’s syndrome

Question 16

What are the clinical features of Cushing’s syndrome?

Answer 16

Amenorrhea, strifes, hyperpigmentation, central obesity, HTN, proximal muscle wasting, signs of androgen excess, and emotional lability

Question 17

What are the two different types of cushings disease?

Answer 17

ACTH dependent (80%) and ACTH independent (20%)

Question 18

What is the most common cause of Cushing’s syndrome?

Answer 18

Pituitary hypersecretion of ACTH, responsible for most cases of Cushing syndrome. This is known as cushings disease

Question 19

What is the most common case of ACTH independent Cushing syndrome?

Answer 19

Latrogenic or fictitious Cushing’s syndrome

Question 20

Is ACTH elevated or lowered in ACTH dependent Cushing’s syndrome?
What about ACTH independent?

Answer 20

Dependent: High
Independent: low

Question 21

What is the gold standard for diagnosis of Cushing’s syndrome? What other tests can be done?

Answer 21

24 hour urinary free cortisol excretion is gold standard.

Late night salivary cortisol and low dose dexamethasone suppression test may also be used

Question 22

What must you rule out when diagnosing Cushing’s syndrome?

Answer 22

You must exclude exogenous glucocorticoids

Question 23

How is the lose dose dexamethasone suppression test performed?

Answer 23

1mg oral dose of dexamethasone at 11pm and serum cortisol levels are measured at 8am the following morning

Question 24

What is an abnormal result in the low dose dexamethasone suppression test?

Answer 24

Elevated cortisol of >5mcg/dL is an abnormal result.

This is highly suspicious for non-suppressive cortisol production from ACTH independent etiology

Question 25

What is the management of cushings disease caused by exogenous corticosteroids?

Answer 25

Taper to the lowest therapeutic dose to control symptoms

Question 26

What is the management of Cushing’s syndrome caused by pituitary adenoma?

Answer 26

Transsphenoidal resection

Question 27

What is the management of Cushing’s syndrome caused by adrenal tumor?

Answer 27

Adrenalectomy

Question 28

What is the management of Cushing’s syndrome caused by adrenal hyperplasia, inoperable tumor, or other malignancy?

Answer 28

Medial therapy is warranted

Question 29

What is the first line medical management of Cushing’s syndrome?

Answer 29

Ketoconazole (at higher doses, it inhibits steroidogensis)

Question 30

What drugs should you give for Cushing’s syndrome if ketoconazole was not effective?

Answer 30

Metyrapone

Question 31

What medication can be given for Cushing’s syndrome to achieve a “medical Adrenalectomy”?

Answer 31

Mitotane

Question 32

What are the functions of aldosterone?

Answer 32

Increase blood pressure, increase ECF volume, increase tubular reabsorption of sodium, promote excretion on potassium, and water retention

Question 33

What are the most common etiologies of primary hyperaldosteronism (Conns syndrome)?

Answer 33

- Bilateral idiopathic adrenal hyperplasia is most common | - Unilateral aldosterone producing adenoma is second most common

Question 34

What are the clinical features of primary hyperaldosteronism?

Answer 34

HTN, hypokalemia, muscle weakness, paresthesias, HA, polyuria, and polydipsia

Question 35

How is primary hyperaldosteronism diagnosed?

Answer 35

1) increased plasma aldosterone concentration (PAC) 2) Decreased plasma renin activity (PRA) or plasma renin concentration (PRC) 3) Spontaneous hypokalemia

Question 36

What imaging can be obtained for primary hyperaldosteronism and why?

Answer 36

CT abdomen to evaluate for adrenal adenoma or adrenal hyperplasia

Question 37

If a patient being worked up for primary hyperaldosteronism does not have spontaneous hypokalemia, what must be done?

Answer 37

Confirmatory testing is required to establish diagnosis

Question 38

What is the management for a patient with primary hyperaldosteronism caused by a unilateral adrenal adenoma?

Answer 38

Surgical removal

Question 39

What is the management of a patient with primary hyperaldosteronism caused by bilateral idiopathic adrenal hyperplasia?

Answer 39

- Mineralocorticoid receptor antagonist (Spironolactone is first line!!) - Antihypertensives - Monitor serum K, creatinine, and BP during first 4-6 weeks of therapy

Question 40

What disease results in decreased synthesis of all adrenocortical hormones, including cortisol, aldosterone, and androgens, however ACTH is high?

Answer 40

Primary adrenocortical insufficiency AKA Addison’s disease

Question 41

What causes secondary adrenocortical insufficiency?

Answer 41

Failure of the corticotrophs to adequately secrete ACTH

Question 42

What causes tertiary adrenocortical insuffiency?

Answer 42

Insufficient CRH from the hypothalamus

Question 43

What is the difference between primary adrenocortical insufficiency (AI) vs secondary and tertiary?

Answer 43

Secondary and tertiary AI clinical features are related to cortisol and androgen deficiency. Aldosterone is normal and hyperpigmentation does not occur

Question 44

What is the most common etiology of primary AI?

Answer 44

Autoimmune destruction of the adrenal cortex

Question 45

What is the most common cause of secondary AI?

Answer 45

Suppression of the HPA axis through abrupt cessation of exogenous steroids

Question 46

What is the one labs that is high in primary AI?

Answer 46

ACTH

Question 47

What lab is normal with secondary AI?

Answer 47

Aldosterone

Question 48

What lab is normal and what is lab is low in tertiary AI?

Answer 48

Aldosterone is normal and CRH is low

Question 49

What are the clinical features of primary AI?

Answer 49

Hyperpigmentation, salt craving, postural hypotension, depression/anxiety, and decreased pubic and axillary hair in females

Question 50

How is AI diagnosed?

Answer 50

- Serum AM cortisol - cosyntropin (ACTH) stimulation test - ACTH, renin, and aldosterone

Question 51

What is an abnormal result of the cosyntropin stimulation test?

Answer 51

Cortisol fails to increase by 7mg/dL above baseline, OR to >18mcg/dL

Question 52

What are the management options of AI?

Answer 52

- Short acting glucocorticoids ( Hydrocortisone BID to TID) - Long acting glucocorticoids (dexamethasone, prednisone) - MIneralocorticoid (Fludrocortisone) - Consider oral DHEA replacement in women

Question 53

What is it called when a patient has a catecholamine secreting tumor that arises from chromaffin cells of the adrenal medulla?

Answer 53

Pheochromocytoma

Question 54

What is the clinical presentation of pheochromocytoma?

Answer 54

Classic triad: Episodic headache, tachycardia, and sweating -paroxysmal HTN

Question 55

What are the most common etiologies of pheochromocytoma?

Answer 55

- 60% are sporadic | - 40% are familial

Question 56

Are pheochromocytomas usually malignant or benign?

Answer 56

90% are benign

Question 57

What do most catecholamine secreting tumors arise from?

Answer 57

The adrenal medulla

Question 58

Patient presents with classic paroxysmal “attacks”, refractory HTN, and an abdominal mass. What should you suspect?

Answer 58

Pheochromocytoma

Question 59

How is pheochromocytoma diagnosed?

Answer 59

- Plasma metanephrines - 24 hour urine catecholamines, metanephrines, and VMA - Clonidine suppression test - thyroid function tests - plasma epi and NE

Question 60

What imaging should be ordered to diagnose pheochromocytoma?

Answer 60

CT abdomen without contrast

Question 61

What is the management of pheochromocytoma?

Answer 61

- Chemical sympathectomy stabilizes patient until surgery - Surgery is definitive - B blocker and alpha blocker

Question 62

What is it called when a patient has an adrenal mass lesion greater than 1cm in diameter, serendipitously discovered by radiologic exam?

Answer 62

Adrenal incidentaloma

Question 63

What is the management for an adrenal incidentaloma that has a negative work up, is likely benign, and is <2cm?

Answer 63

Repeat imaging at 6 months and repeat dexamethasone supression test every year for 4 years

Question 64

What is the management for an adrenal incidentaloma that has a negative work up, is likely benign, and is >2cm?

Answer 64

Consider surgical resection