Adrenal Gland Disorders Flashcards

1
Q

bp guidelines in ph

A

> 140 systolic

>90 diastolic

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2
Q

when to screen for secondary hpn

A

drug-resistance (uncontrolled bp with at least 3 drugs, 1 being diuretic)
abrupt onset
young age
excessive target end organ damage
diastolic hpn
unprovoked/excessive hypokalemia with elevated bp

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3
Q

____ causes predominate in secondary hpn causes

A

adrenal dependent

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4
Q

adrenal cortical hormones

A

mineralocorticoids: aldosterone
glucocorticoids: cortisol
sex hormonres: testosterone -> dht or estradiol

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5
Q

adrenal medullary hormones

A

epinephrine and norepinephrine (degraded by mao and comt)

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6
Q

hormones that can cause hpn

A

mineralocorticoids
glucocorticoids
catecholamines
(all except sex)

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7
Q

t/f acth only acts on mineralocorticoids

A

f, it acts on the entire adrenal cortex. not just cortisol that will be produced when acth is secreted (also other hormones and metabolites)

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8
Q

mechanisms of hpn in cushing’s

A
  • increased production of 11-deoxycorticosterone
  • enhanced pressor sensitivity to endogenous vasoconstrictors
  • increased co
  • activation of the raas by the increased hepatic production of angiotensinogen
  • cortisol activation of the mineralocorticoid receptor
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9
Q

how to workup cushings

A

screening
confirmation of diagnosis
classification

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10
Q

who to screen for cushings?

A

people with hpn

people with clinical manifestations of co-morbid conditions associated with cushing’s

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11
Q

clinical presentations of cushing’s

A

fat-related
cutaneous
muscular
emotional and cognitive

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12
Q

screening tests for cushing’s

A

24 hour urinary free cortisol
overnight 1 mg dexamethasone suppression
late night salivary cortisol

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13
Q

normal or abnormal results for screening tests for cushings

A

24h urinary: levels > 3-4x upper limit of normal is suggestive of cushings
overnight 1 mg dexamethasone: normal should suppress plasma cortisol to <1.8 mcg/dl or 50 nmol/dl
saliva: normal is <145 ng/dl or < 4nmol/dl

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14
Q

conditions associated with hypercortisolism with clinical features of cushings

A
pregnancy
depression or other psych
alcohol dependance
morbid obesity
poorly controlled dm
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15
Q

causes of acth dependent cushing’s

A

pituitary adenoma

non-pituitary neoplasm (ectopic)

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16
Q

causes of acth independent cushing’s

A

iatrogenic: glucocorticoid intake and megestrol acetate intake
adrenal neoplasm
nodular adrenal hyperplasia
factitious

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17
Q

most common cause of cushing’s

A

pituitary adenoma (acth dependent)

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18
Q

initial imaging studies for cushings

A

abdominal ct for primary adrenal problem

pituitary mri for pituitary source

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19
Q

specialized imaging studies for cushings

A

inferior petrosal sinus sampling study: for lateralizing occult lesion in the pituitary and guiding surgical therapy
chest and abdominal ct for suspected ectopic acth

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20
Q

test done after confirming cushing’s diagnosis

A

check serum acth

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21
Q

results of acth tests for cushings

A

acth suppressed: acth independent cushing from adrenal glands
acth is normal/high: acth dependent cushings from pituitary or ectopic source

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22
Q

how to differentiate pituitary vs ectopic source

A

high dose dexamethasone suppression test

suppressed: pituitary
not suppressed: ectopic

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23
Q

last resort test for cushings diagnosis

A

inferior petrosal sinus sampling

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24
Q

initial treatment of choice for cushings

A

surgical resection (TSS)

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25
Q

second line treatment for cushings

A

medical treatment with or without radiation

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26
Q

medical treatment in cushings is primary for

A

ectopic autonomous secretion of cortisol in patients with occult or metastatic eas

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27
Q

medical treatment is adjunctive therapy for

A

adrenocortical carcinoma, to reduce cortisol levels

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28
Q

indications for radiation therapy in cushings

A

concerns about mass effects or invasion associated with corticotroph adenomas
patients who have failed tss or recurrent cushing’s

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29
Q

t/f you need to confirm that medical therapy is effective in normalizing cortisol before administering radiation therapy

A

true

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30
Q

treatment for acth dependent cushings, ectopic and no tumors

A

steroidogenesis i
gc receptor antagonist
bilateral adrenalectomy

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31
Q

treatment for acth dependent cushings, pituitary source or >6 mm mass

A

tumor resection via tss

if failed, repeat tss, pituitary directed medical treatment, or radiation treatment and steroidogenesis inhibitors

32
Q

treatment for acth independent cushing’s

A

bilateral/unilateral adrenalectomy and treat metastatic disease

33
Q

effects angiotensin II

A

causes vasoconstriction of arterioles
stimulation of adrenal cortex to release aldosterone (inc sodium water reabsorption, inc secretion of potassium to urine)

34
Q

the most common specifically treatable and potentially curable form of hpn

A

primary aldosteronism

35
Q

clinical presentation of primary aldosteronism

A
inc aldosterone secretion
inc renal sodium reabsorption
extracellular fluid volume expansion
suppression of renin
hypertension
hypokalemia
36
Q

screening test for primary aldosteronism

A

plasma renin/aldosterone ratio

PAC:PRA ratio >30 = investigate for primary aldosteronism

37
Q

indications for screening for primary aldosteronism

A
sustained bp 150/90
resistant hypertension
bp controlled with 4 drugs
spontaneous
adrenal incidentaloma
obstructive sleep apnea
family history
cva at young age
first degree relative with primary aldosteronism
38
Q

false positives from ARR

A

impaired renal function
advancing age
medicaitons (b adrenergic blockers, methyldopa, clonidine, nsaids)

39
Q

false negatives from ARR

A

hypokalemia
dietary salt restriction
pregnancy
medications (diuretics, CCB, ACEI, ARB)

40
Q

confirmation tests for primary aldosteronism

A

iv saline suppression

captopril challenge test

41
Q

results for iv saline suppression

A

measures pac baseline and 4h

normal: <5 ng/dl at 4h
indeterminate: 5-19 ng/dl at 4h
abnormal: >/= 15 ng/dl at baseline and >/= 10 ng/dl at 4h

42
Q

results of captopril challenge test

A

measures pac baseline and 1-2 h

normal: pac suppressed by 30%
abnormal: no suppression

43
Q

subtypes of primary aldosteronism

A

bilateral idiopathic adrenal hyperplasia or idiopathic hyperaldosteronism*
aldosterone producing adenoma

44
Q

gold standard test for subtype classification

A

adrenal venous sampling

45
Q

other tests for subtype classification

A

adrenal scintigraphy

adrenal ct and mri

46
Q

algorithm for PA

A

hypertension w/ risk factor -> arr -> arr >30 -> confirmatory test -> subtype classification

47
Q

what do you do when patient is positive for ARR and unwilling to proceed with tests

A

treat with mineralocorticoid receptor antagonist

48
Q

indications to proceed with MR antagonist

A

very low potassium with very low renin and >20 ng/dl PAC

if patient doesnt want to undergo surgery

49
Q

treatment if AVS shows bilateral adrenal hyperplasia

A

surgery with MR antagonist

or MR antagonist only (if surgery not desired)

50
Q

treatment if AVS shows unilateral adrenal mass

A

laparoscopic adrenalectomy

51
Q

criteria for laparoscopic adrenalectomy

A

marked PA
young age
positive in adrenal ct
unilateral adrenal mass

52
Q

MR antagonists

A

spironolactone - available in ph

eplerenone

53
Q

other drugs for PA

A

amiloride: aldosterone independent antagonist of renal tubular sodium transport
CCBs
ACEi blocks intraadrenal raas

54
Q

after successful laparoscopic adrenalectomy in a unilateral mass, patients will be normotensive even after ____

A

5 years

55
Q

___ and ___ are tumors that produce catecholamines

A

pheochromocytoma (intra adrenal) and paraganglioma (extra adrenal)

56
Q

when to screen for ppgl?

A
paroxysmal symptoms
paradoxic bp responses to certain medication
resistant hpn
incidental adrenal mass
previous ppgl
family history
syndromic features
57
Q

most common symptoms in ppgl

A

headache
diaphoresis
forceful heartbeat

58
Q

ppgl biochemical phenotypes, adrenergic

A

adrenal medulla
associated with kinase pathway mutations
more paroxysmal symptoms

59
Q

ppgl biochemical phenotypes, noradrenergic

A

adrenal medulla or extra renal
associated with hypoxic signaling pathway mutations
less paroxysmal symptoms

60
Q

medications that can trigger ppgl crisis

A
d2 receptor antagonists
b-adrenergic receptor blockers
sympathomimetics
opioid analgesics
NE reuptake inhibitors
SSRI
MAO inhibitors
corticosteroids
peptides
neuromuscular blocking agents
61
Q

syndromes associated with ppgl

A

MEN 2a/2b
VHL syndrome
NF 1

62
Q

metabolites measured in ppgl confirmation

A

fractionated catecholamines

fractionated metanephrines

63
Q

tests for ppgl confirmation

A

plasma free metanephrines

24h urinary fractionated metanephrines

64
Q

drugs that can cause false positive ppgl

A

mao-i, sympathomimetics, cocaine, levodopa, b blockers

65
Q

plasma free metanephrine results

A

normal: no ppgl

elevated >3x upper limit: highly predictive of ppgl

66
Q

24h urinary fractionated metanephrine results

A

normal: excludes ppgl

elevated >2x upper limit: highly predictive of ppgl

67
Q

when to do imaging for ppgl

A

when there is clear biochemical evidence

68
Q

first choice for ppgl imaging

A

ct scan: thorax abdomen and pelvis

69
Q

other types of imaging for ppgl

A

mri: skull base and neck
mibg scintigraphy: metastatic
pdg pet/ct scan: preferred for metastatic

70
Q

t/f genetic testing is required prior to starting treatment for ppgl

A

false

71
Q

management for ppgl

A

surgical: minimally invasive adrenalectomy or open resection

72
Q

peri-operative treatments before surgery

A

non-pharma: high sodium and fluid intake

pharma

73
Q

pharma peri-operative treatments for ppgl

A

table 6

74
Q

t/f we dont need to follow the sequence when giving presurgical medical prep for ppgl

A

false, bblockers ahead of adrenergic blockers can precipitate a hypertesive crisis

75
Q

measure plasma or urine levels of metanephrines at ___

A

2-4 weeks after surgery

76
Q

t/f lifelong biochemical testing is needed after ppgl surgery

A

true