Adrenal gland disorders Flashcards

0
Q

ACTH independent CYshing SYnd

A

High cortisol but low ACTH (due to negative FB)

1) Adrenal tumor produces high cortisol directly ->negative FB on ACTH so ACTH is low
2) nueroendocrine tumore
3) PHEOchromocytoma

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1
Q

ACTH dependent Cushings Synd

A

High cortisol and high ACTH due to ACTH producing tumor (pit or ectopic)
1) Pit Adenoma is #1 cause - secretes high ACTH -> high cortisol
2) Ectopic ACTH - small cell LUNG CA is #2
but also ovary, pancreatic , thymus CA
3) HypoThalamus produces high CRH -> high ACTH -> high cortisol

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2
Q

Dx work up for cushing syndrome

A

Plasma Cortisol level (looking for high cortisol):
Abnormal is >25 at 8 am and doesn’t drop by 1/3-2/3 at 4pm
(if at 8 am its’ 75, at 4 pm should be 50)

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3
Q

Serum ACTH level in cushing syndrom

A

should be low in ACTH independent
high if ACTH dependent: at 8 am >80, at 4 pm >50 or
low is ACTH indep: <20 at any time

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4
Q

how is 11-deoxycortisol measured and why

A

it’s a precursor to cortisol

measure by radioimmunoassay

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5
Q

Dexamethasone suppression test

A

suppress cortisol with DST (can’t gell ACTH levels)

Abnormal if cortisol > 5 in am -> cushings

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6
Q

24 hr urine free cortisol

A

definitive confirmation
measures 17 OCHS
MAIN STAY for Dx
Abnormal if cortisol >125

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7
Q

what is the gold standard for screening of cushings

A

DST suppression test

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8
Q

what is the main stay for dx of cushing

A

24 hr urine cortisol - confirmation

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9
Q

4 Dx work ups for Cushings synd

A

1) Plasma cortisol (8 am and should decrease by 1/3-2/3 at 4 pm) and Serum ACTH (8 adn 4)
2) Dexamethasone suppression test (Gold standard - only tests cortisol)
3) 24 hr urine for cortisol (definite confirmation of dx)
4) salivery samples bf brushing teath (9 am x 3 days for adrenal insuff and 11 pm x 3 days for Cushings)

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10
Q

Salivary sampling

A

before brush teeth,
9 AM x 3 day for adrenal insuff (cortisol should be high, if low -> adrenal insuff)
11 pm x 3 day for Cushings ( cortisol should be low, if high -> cushings)

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11
Q

How to Dx cushings

A

1) exclude exogenous use of cortisol (for autoimmune dz, ashthma)
2) Is cushings due to pituitary tumor (ACTH dep’t)
3) Is it due to ectopic ACTH producing tumor?
- get clinical clue from hx, maybe CXR to rule out lung mass ((Small cell LUNG CA #2 cause of ACTH dept, pituitary adenoma is #1), PELVIC U/S for OVARIAN CA
4) Is it adrenal tumor (ACTH indep’t/ high cortisol -> low ACTH):
- palpate abdomen for masses
- maybe CT abdomen

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12
Q

If cushings due to pituitary adenoma - what tx

A

pit adenoma (ACTH dept) - do trans-sphenoidal resection (ENT surgery)

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13
Q

If adrenal tumor causing cushings

A

(ACTH indept) - do adrenalectomy (general surgery)

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14
Q

if exogenous corticoid use is the cause of cushings

A

taper to lowest effective dose that can tx pt’s sx

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15
Q

if adrenal hyperplasia (ACTH independent) or inoperable ectopic

A

do medical tx - oncology

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16
Q

Most common cause of cushings is

A

ACTH producing pituitary adenoma

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17
Q

most common cause of cushingoid sx is

A

exogenous stroid use (but not tumor, why if adenoma is #1 cause?)

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18
Q

Sx of cushings

A

Central obesity, stretch marks, moon face
Spontaneous ecchymosis (bruises), virilization
unexplained OSTEOPOROSIS, HTN
NEW onset insulin resistance/DM

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19
Q

Cushings Tx

A

Mitotane - kills cortisol producing cell
Kenoconazole - antifungal in high dose but major SE: liver tox
Metyrapone - inhibits cortisol synthesis

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20
Q

what is the major SE of ketokonazole

A

LIVER TOXicity

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21
Q

Mitotane is used to tx which disorder

A

Cushings

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22
Q

Metyrapone is use to tx what

A

cushings

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23
Q

ketoconazole is used to tx what

A

cushings

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24
what is the first line tx for cushings
surgery
25
which test is used for definitive dx of cushings?
24 hr urine cortisol
26
which is the easiest test for cushings
DExamethasone suppression test but for definite dx do 24 hr cortisol urines
27
which test to use to ddx ACTH dept from independent cushings?
Comparison of ACTH and Cortisol levels at 8 and 4 pm
28
Adrenal INsuff
any age, | decreased production of glucocorticoids (cortisol), mineralocorticoids (ALDO) and/or sex androgen (testosterone)
29
Anatomic descruction of adrenal cortex (adrenal gland) can be due to (primary - problem in adrenal gland, secondary - elsewhere and affects gland)
MOst: autoimmune = Addison's Dz (ABs destroy gland) Surgical adrenalectomy Infection (coccidiodomycosis, histoplasmosis, TB disseminated to gland and didn't clear) Hemorrage or Infarct Mets or CA AIDS
30
Metobolic failuer in hormone prod of adrenal gland can be due to
Congenital adr hyperplasia | ALD - adredoleukodystrophy (Susan Serandon)
31
What is the secondary etiology of adr insufficiency
MOSTLY: ABRUPT cessation of exogenous cortisol tx: will suppress both hypoth and pit for about 12 months hypopotuitarism/pit dz stroid producing tumor (why would it not cause increase in cortisol?)
32
which drugs reduce cortisol production
opiates, KETOCONAZOLE (tx for cushings), rifampin, phenytoin
33
Hallmark TETRAD of adr insuff (KNOW!)
Weakness/fatigues (worse with stress) Wgt LOSS/ anorexia (+/- with N,V, abd pain, hypoglycemia t retain H20) - ORTHOSTATIC ALso: salt craving, syncope, loss of pubic hair and amenorrhea
34
4 work ups for adren insuff
1) ACTH vx Cortisol test 2) CoSynTropin Stim Test - comfirmatory dx like 24 hr urine, get baseline cortisol, Give bolus of ACTH, measure cort at 30 and 60 min: if fail to increase by >18 = abnormal 2)Metyrapone Stim Test: don't need to dc steroids, stnd is 3 day: do 24 hr urine baseline, then take metyrapon and collect 24 hr urine on day 2 and 3 measures: creat and 17-OHCS in urine, also measure serium cort and ACTH 4 hrs after last dose 4) check SALIVA cort level in AM (the slide says 9 pm??)
35
Other labs to order in adrenal insuff and why
CMP - bc decreased Aldo = low Na, HyperK, HyperCalcemia CBC bc anemia of chronic dz (normochromin, normocytic) ABs bc most common cause of AI is Addisons (autoimmune) TSH, PRL, FSH LH - bc could have pit dysfx: pituitary is not secreting ACTH (low cortisol), TSH (hypothyroid), FSH/LH (menstrual irreg), Prolactin (what sx are PRL deficiency?)
36
How do you manage Pheo
``` IV Nitro to tx HTN crisis alpha blocker (phenoxybenzamine) poss followed by beta blocker (propranolol): to stabilize pt till definitive therapy Surgery/Excision - definitive tx (always refer to endo and surgery) ```
37
WHat is Pheo
Tumor on Adrenal gland that produces catecholamines (Epi, NE): HA, Sweat, Palpitations for 30-40 min (panic like or hyperthyroid-like) Acute HTN crisis (use IV nitro) (refractory HTN) NEVER USE FNA (Fine needle aspiration) worse when bend over or palpate abdomen
38
When is IV nitro used
for Pheo in acute HTN crisis
39
When do you NEVER use FNA?
in Pheo
40
What causes elevated Epi and NE (cats)
Pheo- cats-producing adrenal tumor
41
What is the first test to run when suspect Pheo
Thyroid panel to see if hyperthyroid bc sx are similar: palpitations, sweating, HTN, and HA (no HA in thyroid) -also do plasma EPi and NE and 24 hr urine for cats and metanephrines (urine better than plasma) Pt has to be supine for 30 min with needle inside
42
WHich drugs could cause False positive for Pheo and why
``` W/D of clonidine (Alpha 2 agonist decreases SNS (Epi and NE) so sudden WD will sky rocket Epi and NE Beta blockers Alcohol Tylenol Tricyclics ```
43
What is the test used for definitive Dx of Pheo?
Clonidine suppression test: if you have normal Epi and NE levels, then clonidine should suppress it 3 hrs after admin (bc alpha 2 blocks SNS) if you have abnormally high Epi and NE, then they will not still be high!
44
what is special ab clonidine test?
must stop all hypotensive meds for 24 hrs and fast overnight then take baseline BP and pulse draw labs at 3 hr intervals with BP and pulse
45
Would you do and MRI or CT on PHeo
yes because want to remove tumor
46
Adrenal Incidentaloma
Mass lesion > 1 cm discovered by incident: abdominal (most non-functioning) is it malignant? -> U/S , CT is it functioning? -> secretes (if on adrenals -> will have high cortisol)
47
Work up for indicentalomas
1) EVERYONE: check plasma cortisol, ACTH (pituitary or adrenal?), DHEA (adrenal androgens), adlosterone (Conn syndrome - hyperAldo, secreted by pituitary?) 2) if have sx of cushings (central obesity, buffalo hump, HTN stretch marks) - do a 24 hr urine for free cortisol if no sx of cushings - do an overnight Dexamethasone suppression test (screening for cushings) 3) if suspect Pheo (high Epi, NE): - first do Hx and physical: HA, palpitations, sweating acute HTN for 30-40 min - 24 hr urine cats and metanephrines - serum metanephrines
48
when to NOT use fine needle biopsy
when have biochemical proof of Pheo known widespread mets Do use: when known primary malignancy elsewhere
49
Based on work up findings
if pheo or CA - surgery pharma for underlying dx if benign appearance of imaging: >2 cm -> consider resectin repeat imaging in 6 months (CT usu better or MRI) REPEAT DEXAMETH SUPPR test each year for 4 years
50
What must one always r/o with adrenal insuff?
TB!!!! (lung, breast CA and vascular dz may impair adrenocortical function -screen with CXR and mammogram if in high risk category)
51
When to do radiographic studies
not when it's autoimmune CT can ID infection, hemorrhage, CA MRI - 2ndary adrenal insuff (low ACTH and low Cortisol)
52
TX of Adr insuff
1) Glucocorticoid replacement: hydrocortisone, prednisone, dexamethasone 2) Mineralocorticoid replacement (for primary adren ins)" Fludrocortisone 3)Adrenal androgen replacement if needed: DHEA
53
What must you always tell a pt with adr insuf
ALways increase your glucocorticoids whe highly stressful events
54
what is the gold standard for Adrenal Insuf DX?
Cosyntropin stimulation test
55
what is conn synderome
HyperAldosteroism
56
HyperAldo (Conn) is due to
adrenocortical adenoma (70%) or cortical hyperplasia (30%) - similar to ACTH independent cushings syndrome
57
HyperAldo sx
???High Aldo= HIgh Na, low K, HTN -> low RENIN Sx: HTN, hypoK, paresthesia, HA, polyuria and polidypsia (like DM - misdx?) why polyuria???
58
Tx of Conn/HyperAldo
surgery | spironolactone and antiHTN (to supress till surgery only?)
59
Dx work up for HyperAldo:
High plasma and urine Aldo Low plasma RENIN CT adrenals to eval for adenoma