The Adrenal Gland

Question 1

Short term stress results in release of _________ from the adrenals, while long term stress results in _____ and _____ release from the adrenals

Answer 1

Catecholamines; mineralocorticoids; glucocorticoids

Question 2

Layers of adrenal cortex and their hormones

Answer 2

Glomerulosa — mineralocorticoids

Fasciculata — glucocorticoids

Reticularis — sex steroids

Question 3

What hormones are elevated in 21-hydroxylase deficiency?

Answer 3

DHEA
Androstenedione
Testosterone

Question 4

Treatment for 21-hydroxylase deficiency

Answer 4

Hydrocortisone
Fluids/glucose
Get K+ level down

Question 5

T/F: congenital adrenal hyperplasia is one of the disorders included in newborn screening

Answer 5

True

Question 6

Most common cause of primary adrenal insufficiency (addison) in the US

Answer 6

Autoimmune

2nd most common cause is TB

Question 7

Cause of primary adrenal insufficiency that involves bilateral hemorrhage of the adrenals

Answer 7

Waterhouse-Friderichsen syndrome

Question 8

Describe inheritance and presentation of familial glucocorticoid deficiency

Answer 8

Autosomal Recessive

Inherited adrenal unresponsiveness to ACTH —> increase in ACTH results in skin pigmentation

Question 9

Most common type of congenital adrenal hyperplasia

Answer 9

21-hydroxylase deficiency

Presents with salt wasting (aldosterone deficiency), deficiency of cortisol, virilization in females (excess androgens)

Question 10

Clinical signs/symptoms of primary adrenal insufficiency

Answer 10

Nearly all pts complain of fatigue, reduced stamina, weakness, anorexia, weight loss, and skin hyperpigmentation

Some pts may complain of abd pain, N/V, MSK pain, psychiatric sxs, HA, gait disturbance, salt craving, orthostatic light headedness, virilization in females

Question 11

Lab findings in primary adrenal insufficiency

Answer 11

Low serum Na
High serum K+
Fasting hypoglycemia
Hypercalcemia

LOW 8AM PLASMA CORTISOL + SIGNIFICANT ELEVATION OF PLASMA ACTH

Question 12

What test is used to confirm primary adrenal insufficiency?

Answer 12

Cosyntropin (synthetic ACTH) stimulation test

If cortisol levels do not respond to cosyntropin, primary adrenal insufficiency is confirmed

Question 13

How is a dx of autoimmune addison disease confirmed?

Answer 13

Serum Abs to 21-hydroxylase

Question 14

In 21-hydroxylase deficiency, elevated serum levels of ______ are required for dx

Answer 14

17-OH-progesterone

Question 15

Treatment for primary adrenal insufficiency

Answer 15

Glucocorticoid replacement therapy

Mineralocorticoid replacement therapy

DHEA — given to some postmenopausal women

Question 16

Low serum ACTH in the setting of low serum cortisol indicates __________

Answer 16

Secondary adrenal insufficiency

Question 17

Cushing disease is considered ACTH-_______ Cushing syndrome

Exogenous corticosteroid use, adrenocortical tumor, and McCune Albright syndrome are examples of ACTH-_______ Cushing syndrome

Answer 17

Dependent

Independent

Question 18

Describe the Dexamethasone suppression test for Cushing syndrome

Answer 18

Low dose dexamethasone given at night, serum cortisol drawn in the morning

If the serum cortisol is below a certain level, Cushing syndrome is excluded with some certainty. Result is then used in conjunction with ACTH levels (drawn before dexamethasone is given) to determine the cause of the hypercortisolism

Question 19

Results of dexamethasone suppression test that indicate ectopic ACTH syndrome

Answer 19

Elevated ACTH and cortisol is not suppressed by high or low doses

Question 20

What do most pts with primary hyperaldosteronism present with?

Answer 20

Hypertension

Metabolic alkalosis (proton loss, H+ with K+)

[may see hypokalemia in 37%]

Question 21

What is Conn syndrome

Answer 21

Primary aldosteronism secondary to a unilateral aldosterone-producing adrenal adenoma

40% have a mutation in a gene coding for the K+ channel

Question 22

What disease is associated with 20% developing pheochromocytomas (NE-secreting), hemangiomas, hyperthyroidism, pancreatic cysts, endolymphatic sac tumors, cystadenomas of adnexa or epididymis, pancreatic neuroendocrine tumors, renal cysts, adenomas, and carcinomas?

Answer 22

Von Hippel-Lindau disease type 2 (autosomal dominant)

Question 23

Lab findings in pheochromocytoma

Answer 23

Plasma fractionated free metanephrins is the single most sensitive test for secretory pheochromocytomas and paragangliomas

Question 24

Medical treatment prior to resection of pheochromocytomas

Answer 24

Alpha-blockers — Phenoxybenzamine is a long acting nonselective alpha blocker

Also give Ca-channel blockers, and beta-blocker (usually metoprolol)

Question 25

Inheritence and significance of MEN syndromes

Answer 25

Autosomal dominant

Cause a predisposition to the development of tumors in 2+ endocrine glands; MEN 2 and 3 both associated with pheochromocytomas