7.31 B Adrenal Medulla

Question 1

neuroendocrine cells and sustentacular cells

• what organ
• secrete what

Answer 1

adrenal medulla

epinephrine and norepinephrine

Question 2

tumor of chromaffin cells

• is called
• causes what bodily effect

Answer 2

pheochromocytoma

hypertension

Question 3

patient with HTN and salt and pepper chromatin

Answer 3

pheochromocytoma

Question 4

familial syndromes associated w/ pheos and paragangliomas

Answer 4

MEN2A

MEN2B

Neurofibromatosis 1

Von Hippel-Lindau

Familial paraganglioma

Question 5

lab diagnosis of pheo

Answer 5

increased urinary excretion of free catecholamines and their metabolites

• metanephrine
• vanillylmandelic acid (VMA)

Question 6

treatment of pheo

Answer 6

surgical excision if isolated

anti hypertensives if multifocal

Question 7

carotid body tumor is a type of

Answer 7

paraganglioma

Question 8

never do FNA if you suspect

Answer 8

pheo

Question 9

aldosterone regulates

Answer 9

salt

Question 10

chronic increase in cortisol

- dx

Answer 10

Cushing syndrome

Question 11

bilateral hyperplasia of adrenal gland

- cause

Answer 11

• elevated serum ACTH from anterior pituitary

- paraneoplastic (SCC of lung)

Question 12

unilateral hyperplasia of adrenal gland

• cause
• mechanism

Answer 12

primary adrenal adenoma or carcinoma

secretes ACTH, which increases cortisol, which suppresses natural ACTH

Question 13

ACTH-producing pituitary micro adenoma causes increased serum ACTH and subsequent increase in serum cortisol
- diagnosis

Answer 13

Cushing disease / pituitary hyper secretion of ACTH

Question 14

increased serum cortisol, decreased ACTH

Answer 14

primary adrenal neoplasms (adenomas, carcinomas) / ACTH independent Cushing syndrome

Question 15

hairy woman with depression and diabetes

Answer 15

Cushing syndrome

Question 16

how to diagnose hypercortisolism

Answer 16

increase 24 hour free urine cortisol

loss of diurnal pattern of cortisol secretion (cortisol even high at night)

Question 17

test to discern cause of hypercortisolism

Answer 17

dexamethasone suppression test

Question 18

increased ACTH, no dexamethasone suppression at low dose, but suppressed at high dose

Answer 18

pituitary / Cushing’s disease

Question 19

increase ACTH, no suppression of cortisol with dexamethasone

Answer 19

ectopic ACTH production

Question 20

decrease ACTH, no suppression of cortisol with dexamethasone

Answer 20

adrenal secretion of cortisol

Question 21

patient that sodium retention and potassium excretion, HTN

Answer 21

hyperaldosteronism

Question 22

mechanism of primary hyperaldosteronism

Answer 22

excess aldosterone

suppress renin angiotensin system

decrease plasma renin

sodium retention

increase blood pressure

Question 23

causes of primary hyperaldosteronism

Answer 23

bilateral idiopathic hyperaldosteronism

adrenal cortical neoplasm (Conn syndrome)

glucocorticoid-suppressible (hybrid glomerulosa responsive to ACTH)

Question 24

lab confirmation of primary hyperaldosteronism

Answer 24

increase aldosterone relative to plasma renin

if pos, do aldosteron suppression test

• captopril (ACEi) if pos, won’t stop aldo
• salt loading suppression test - if pos, salt still high

Question 25

cause of secondary hyperaldosteronism

Answer 25

activated renin-angiotensin system increase plasma renin increase AT increase aldosterone

Question 26

what activates the renin angiotensin system

Answer 26

decrease renal perfusion CHF cirrhosis nephrotic syndrome pregnancy

Question 27

treatment of hyperaldosteronism if caused by adenoma

Answer 27

surgical removal

Question 28

treatment of hyperaldosteronism if caused by hyperplasia

Answer 28

aldosterone antagonist such as spironolactone

Question 29

adrenal causes of androgen excess

Answer 29

adrenocortical neoplasms congenital adrenal hyperplasia

Question 30

virilization - more likely to be

Answer 30

androgen secreting carcinomas (vs adenomas)

Question 31

can't make aldosterone or cortisol, so increase synthesis of androgens causing virilization, adrenal hyperplasia from decreased cortisol, and salt wasting from decreased aldosterone - dx - inheritance - most likely enzyme deficiency

Answer 31

congenital adrenal hyperplasia autosomal recessive 21 hydroxylase deficiency

Question 32

bilateral adrenal hyperplasia salt wasting virilization (females have phallic genitalia, males have salt wasting)

Answer 32

salt wasting classic syndrome (total deficiency of 21 hydroxylase)

Question 33

just have virilization

Answer 33

simple virilizaing adrenogenital syndrome without salt wasting

Question 34

hirsutism

Answer 34

non classic adrenal virilism

Question 35

2 types of primary adrenocortical insufficiency

Answer 35

acute: waterhouse friedrichsen syndrome chronic: Addison disease

Question 36

DIC, purpura, hypotension, shock, adrenal hemorrhage - diagnosis - cause - treatment

Answer 36

Waterhouse-Friedrichsen syndrome Neisseria meningitidis antibiotic

Question 37

fatigue, anorexia, NVD, hyperpigmentation, hypotension, hyperkalemia, acidosis - diagnosis - causes - lab diagnosis

Answer 37

Addison disease (destruction of adrenal glands) - autoimmune adrenalitis - TB - AIDS - metastatic carcinoma - increase ACTH - if give ACTH, no cortisol response because adrenal cortex is destroyed

Question 38

autoimmune destruction of steroidogenic cells and steroidogenic enzymes like 21 hydroxylase and 17 hydroxylase

Answer 38

autoimmune adrenalitis | - autoimmune polyendocrine syndrome (APS) 1 and 2

Question 39

fatigue, anorexia, NVD - diagnosis - causes - lab diagnosis

Answer 39

secondary adrenocortical insufficiency - disorders of hypothalamus or pituitary - prolonged administration of exogenous glucocorticoids - decreased ACTH - if administer ACTH, increase plasma cortisol

Question 40

virilizing neoplasm more likely to be adenoma or carcinoma

Answer 40

carcinoma

Question 41

functional adenomas most commonly associated with

Answer 41

hyperaldosteronism Cushing (hypercortisolism)

Question 42

Wermer syndrome also called

Answer 42

MEN 1

Question 43

Sipple syndrome also called

Answer 43

MEN2 / MEN2A

Question 44

MEN-1 - organs - mutation & protein

Answer 44

- pituitary (prolactinoma) - parathyroid (primary hyperparathyroidism) - pancreas (gastrinoma from Zollinger-Ellison, hypoglycemia from insulinoma) MEN1 gene for menin

Question 45

kidney stones and stomach ulcers

Answer 45

MEN1

Question 46

MEN2A - organs - mutation

Answer 46

- adrenal pheochromocytoma - medullary thyroid carcinoma - parathyroid hyperplasia - RET proto-oncogene mutation (diff than MEN2B)

Question 47

MEN2B - organs - mutation

Answer 47

- adrenal pheochromocytoma - medullary thyroid carcinoma - mucosal neuromas of skin, GI, resp tct - marfanoid habitus - RET proto oncogene (diff than MEN2A)

Question 48

MEN with the most aggressive medullary thyroid carcinoma

Answer 48

MEN2B

Question 49

if have medullary thyroid carcinoma, which MEN syndrome requires screening for families

Answer 49

MEN2 A and B (not MEN1!)