adrenal HTN: hyperaldosteronism and pheochromocytoma

Question 1

Aldosterone acts primarily on the __________ to increase the reabsorption of Na+ and Cl- ions and the secretion of K+ and H+ ions.

Answer 1

distal nephron

Question 2

Secretion of aldosterone is normally regulated primarily by the

Answer 2

renin-angiotensin system

Question 3

Aldosterone is a mineralocorticoid hormone produced by the cells of the

Answer 3

zona glomerulosa in the adrenal cortex.

Question 4

Primary _________ is a term for adrenal disorders which result from the renin-independent overproduction of aldosterone. The result is hypertension (high blood pressure) and in most cases hypokalemia.

Answer 4

aldosteronism

Question 5

Why polyuria in primary hyperladosteronism?

Answer 5

Hypokalemia can cause impairment of urinary concentrating ability

Question 6

Renal loss of hydrogen ions occurs when excess _____________ increases the activity of a sodium-hydrogen exchange protein in the kidney

Answer 6

aldosterone (Conn’s syndrome)

Question 7

PAC/PRA > ____ is highly suggestive of Primary Aldosteronism

Answer 7

20

Question 8

what happens if we give sodium to patients have high aldosterone levels?

Answer 8

the high salt would normalize the aldosterone levels thus this is used to confirm if the patient has primary hyperaldosteronism

Question 9

Gold Standard for determining etiology of Primary Aldosteronism

Answer 9

adrenal vein sampling

Question 10

adrenal vein sampling that lateralizes

Answer 10

adenoma

Question 11

adrenal vein sampling that is symmetric

Answer 11

hyperplasia

Question 12

Unilateral causes of hyperaldo should be treated with a ____________ while IHA should be treated __________

Answer 12

Unilateral causes of hyperaldo should be treated with a laparoscopic adrenalectomy while IHA should be treated medically.

Question 13

IHA first line medication in females is

Answer 13

spironolactone

** we use eplerenone for males to avoid the gymmncomastia in males

Question 14

is a tumor arising from adrenomedullary chromaffin cells that commonly produces one or more catecholamines: epinephrine, norepinephrine, and dopamine.

Answer 14

pheochromocytoma

Question 15

• extra-adrenal tumor of chromaffin or chromaffin-like cells

Answer 15

Paraganglioma

Question 16

pheochromocytoma occurs in sites containing ______ tissue

Answer 16

chromaffin

Question 17

Typical Paroxysm: 5 P’s

Answer 17

Pressure
Pain
Perspiration
Pallor
Palpitation

Question 18

spells

1. precipitants
2. duration

Answer 18

1. Precipitants: diagnostic procedures, anesthesia induction, drugs that block catechol reuptake-TCA/cocaine, childbirth
2. Duration : variable; monthly to multiple times per day; seconds to 60 min

Question 19

AD d/o; phenotype includes pheo (usually bilateral and may be asynchronous) MTC and hyperparathyroidism.
Pheo occurs in 50% of pts with _____ . MTC is usually detected before pheo. Activating mutations in the RET proto-oncogene have been found.

Answer 19

MEN2A

Question 20

AD d/o with age related penetrance. Phenotype consists of pheo (often bilateral), MTC, mucosal neuromas,
Intestinal ganglioneuromatosis and a marfanoid body habitus. Pheo occurs in 50% of pts with _____
Activating mutations in the RET proto-oncogene have been found.

Answer 20

MEN2B.

Question 21

According to this understanding, the free ______________ are produced within adrenal chromaffin cells (or the tumors derived from these cells) by membrane-bound catecholamine O-methyltransferase. Lack of this enzyme in sympathetic nerves, the major site of initial norepinephrine metabolism, means that the O-methylated metabolites are relatively specific markers of chromaffin tumors.

Answer 21

metanephrines

Question 22

________ has a sensitivity of 100% in detecting pheochromocytomas, does not neccesitate contrast and does not expose the patient to ionizing radiation.

Answer 22

MRI

Question 23

______________ scintigraphy may be done. ____________ (diagnostic) is a compound resembling norepinephrine that is taken up by adrenergic tissue. This scan can detect tumors not detected by CT or MRI or multiple tumors when CT or MRI is positive

Answer 23

Iobenguane I-123

Question 24

is more sensitive than iobenguane I-123 and CT/MRI for detection of metastatic disease

Answer 24

FDG-PET — Fludeoxyglucose-positron emission tomography (FDG-PET)

Question 25

management of pheo

Answer 25

Surgical resection treatment of choice, majority benign and result in cure of HTN

Question 26

Pts with refractory HTN and hypokalemia -screen for

Answer 26

hyperaldosteronism

Question 27

In PA excess CV risk was no longer present after treatment of mineralcocorticoid excess

Answer 27

yep

Question 28

Pheochromocytoma is a potentially curable form of htn but undiagnosed fatal - what medication should we use priot to surgery?

Answer 28

alpha blockade and then Beta blockade

Question 29

life long surveillance is imperative in pheo

Answer 29

yep

Question 30

_____________ usually produce catecholamines and are usually clinically functional, while ________________ are usually clinically silent mass lesions

Answer 30

Sympathetic PGL usually produce catecholamines and are usually clinically functional, while parasympathetic PGL are usually clinically silent mass lesions

Question 31

There is no morphological way to classify a PCC or PGL as malignant or benign. Malignancy is defined by

Answer 31

the development of metastases to sites where normal paraganglia are not present

Question 32

Classic histology for pheo/paraganglioma cells is

Answer 32

balls of cells with peripheral small vessels Zellballen

Question 33

tumor location for pheo is dependent on

Answer 33

specific mutated gene

Question 34

common pheo mutation

Answer 34

succinate Dehydrogenase gene mutations

Question 35

Sympathetic plus parasympathetic paraganglioma VERY suggestive of __________ mutation;

Answer 35

SDHB or SDHD

Question 36

SDHC mutation (PGL3) tumors almost always

Answer 36

parasympathetic