Hyperaldosteronism and Pheochromocytoma Flashcards
where in adrenal do aldosteronomas arise?
in zona glomerulosa
where is issue in primary hyperaldo?
in adrenal gland
what are the two main causes of primary hyperaldo?
unilateral adenoma
and
bilateral adrenal hyperplasia
what is another name for primary hyperaldo from unilateral adenoma?
Conns syndrome
primary hyperaldo leads to what three ion changes
Na reabsorption in kidney
K+ excretion
H+ excretion
what three things do you measure in diagnosis for primary hyperaldo
serum aldo
plasma renin
aldo:renin ration
primary hyperaldo has what lab values for serum aldo
plasma renin
aldo:renin ratio?
Plasma aldo increased
renin decreased
ration increased
secondary hyperaldo has what lab values for serum aldo
plasma renin
aldo:renin ratio?
increased aldo
increased renin
normal or low ratio
what are four possible etiologies of secondary hyperaldo?
renin producing tumor
coarctation of the aorta
fibromuscular dysplasia
renal artery stenosis
if you have what seems to be high aldo but low aldo conc and low renin, what are possible causes? what is this called?
called pseudohyperaldo
Congen adrenal hyperplasia, cushings, licorice are possible causes
what is confirmation test for primary hyperaldo?
aldosterone suppression test by giving a salt load
what should response be to a salt load? what happens in primary hyperalso?
decreased aldosterone…primary hyperaldo does not decrease the aldo in response to increased salt load
what is rx for young patient with clear unilateral adrenal mass secreting aldo?
surgery…unilateral adrenalectomy
what test can you do to help determine where aldo is coming from in adrenals? like which one or both producing?
adrenal vein sampling
if adrenal vein sampling for aldo lateralizes what does this mean?
that aldo increase is mainly from lateralized side and likely a unilateral problem so surgery more likely to help
if adrenal vein sampling does not lateralize what does that mean?
patient likely has bilateral adrenal hyperplasia and aldo coming from both sides
which adrenal vein is easier to sample from? why?
left suprarenal vein because angle is less severe, right is at 90 degree angle
what is rx for aldo producing adenoma?
surgery
what is rx for bilateral hyperplasia?
medication
name the two drugs to treat aldo excess
spiranolactone and epleronone
which of spiranolactone and epleronone is used more, why?
spironolactone…bc cheaper
secondary hyperaldo in adults is most commonly due to what?
renal artery stenosis
what is rx for renal artery stenosis?
balloon angioplasty or ACE/ARBs
what is MEN1?
a genetic neuroendocrine causing tumor
what three tumors arise from MEN1 mutations?
primary hyperparathyroid
pituitary adenomas
pancreatic neuroendocrine tumor
what is inheritance of MEN1
AD
in addition to parathyroid, pancreas and pituitary, what other endo organ can have issue in MEN1?
adrenal adenomas
what happens in MEN 2 type 2A?
medullary thyroid cancer with primary hyperparathyroid
pheochromocytoma
what is inheritance of MEN2 type 2A?
AD
what happens in MEN2 type 2B?
medullary thyroid cancer, pheochromocytoma
what is inheritance of MEN2 type 2b?
AD
what is pheochromocytoma a tumor of?
chromaffin cells in adrenal medulla
why are pheochromocytomas called the 10% tumor?
10% bilateral
10% outside the adrenal medulla in symp ganglia
10% malignant
30% inherited
what are the three main symptoms of pheochromocytoma?
HA
diaphoresis
palpitations
what are two things to measure when worried about pheochromocytoma?
24 hr urine metaneprines and plasma metanephrines
are pheochromocytomas always only in adrenal medulla?
no can have tumor in sympathetic ganglia too. called paraganglionoma
if worried about paraganglionoma what should you do?
CT/MRI of abdomen called 123 I_MIBG
what is fist rx for pheochromocytoma?
surgery
describe medical management of pheochromocytoma
alpha block first then beta block with vigorous hydration
why do you alpha block before you beta block in pheochromocytoma?
beta adrenergic normal effect on vasculature is to vasodilate so if you block it first then alpha is unopposed in constriction and can have worsening HTN
