adrenal disorders 3 - hyperaldosteronism and pheochromocytoma

Question 1

Licorice inhibits the enzyme ______________ in the kidney (which normally inactivates cortisol) resulting in inappropriate stimulation of the mineralocorticoid receptor by the excess cortisol.

Answer 1

11-beta hydroxysteroid dehydrogenase type II (Protein:HSD11B2) enzyme

Question 2

3 layers of the cortex

Answer 2

glomerulosa, fasiculata, reticularis

Question 3

Zona glomerulosa is responsive to _______

Answer 3

angiotensin II

Question 4

_____ is composed of post-ganglionic sympathetic cells = chromaffin cells which secrete catecholamines into blood.

Answer 4

Medulla

Question 5

2/3 of all hyperaldosteronism are caused by

Answer 5

bilateral adrenal hyperplasia

Question 6

Glucocorticoid Remediable Aldosteronism (GRA)

Answer 6

aberrantly formed gene product in the promotor of 11-beta hydroxyls gene and coding region of aldosterone synthetase gene

Question 7

ultimate end result of glucocorticoid remediable aldosteronism (GRA)

Answer 7

aldosterone secretion is controlled by ACTH

Question 8

Conn syndrome

Answer 8

unilateral adenoma causing primary hyperaldosteronism

Question 9

Glucocorticoid Remediable Aldosteronism (GRA) method of inheritance

Answer 9

Type 1 familial primary aldosteronism, auto. dominant

Question 10

in a unilateral adrenal adenoma, low ___ levels do not result in lower aldosterone levels

Answer 10

renin

Question 11

2 causes of primary hyperaldosteronism

Answer 11

unilateral adrenal adenoma (Conn syndrome), and bilateral adrenal hyperplasia (BAH)

Question 12

lab features of PHA

Answer 12

hypertension (swelling, edema),
hypokalemia,
metabolic alkalosis

Question 13

what to screen for in PHA

Answer 13

serum aldosterone, plasma renin, calculation of aldosterone:renin ratio

Question 14

causes of secondary hyperaldosteronism

Answer 14

Renin producing tumor
Coarctation of the aorta
Fibromuscular dysplasia
Renal artery stenosis

Question 15

enzyme that inactivates cortisol in the kidney

Answer 15

11-beta-hydroxysteroid dehydrogenase (type 2)

Question 16

treatment for aldosterone producing adenoma

Answer 16

surgery preferred

Question 17

medications for primary hyperaldosteronism

Answer 17

• aldosterone receptor blockers - spironolactone and epleronone, or other inhibitors of RAAS

Question 18

pheochromocytoma arises from the

Answer 18

medulla

Question 19

MEN1

Answer 19

primary hyperparathyroidism, pituitary adenomas, pancreatic neuroendocrine tumors

Question 20

inheritance of MEN1

Answer 20

autosomal dominant

Question 21

MEN2

Answer 21

Medullary thyroid cancer
Primary hyperparathyroidism
Pheochromocytoma (2a and 2b)

Question 22

inheritance of MEN2

Answer 22

RET gene mutation that is autosomal dominant

Question 23

Malignancy composed of neural crest cells in the adrenal medulla

Answer 23

neuroblastoma

Question 24

neuroblastoma on histology

Answer 24

homer-wright rosettes

Question 25

Neoplasm of chromaffin cells which secrete catecholamines

Answer 25

pheochromocytoma

Question 26

clinical signs of excess catecholamine secretion

Answer 26

hypertension, headache, diaphoresis, palpitations, tremors, anxiety, fatigue

Question 27

diagnosis pheocromocytoma - most specific

Answer 27

increase in 24 rh urine metanephrines

Question 28

Plasma catecholamines and especially metanephrines can be ______ in stressed or sick patients.

Answer 28

falsely elevated