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Flashcards in Adrenal Disorders Deck (34):
1

Primary Adrenal Insufficiency

-low cortisol, aldosterone, and adrenal androgens

Causes:
Addison's Disease (Autoimmune)
Infectious: TB, Fungi, HIV
Infiltrative: Amyloid
Hemorrhage
Metastatic
Metabolic (Adrenoleukodystropy
Adrenomyeloneuropathy)
Surgery

2

Clinical Features of Adrenal insuff

Sx:
Fatigue Anorexia Abdominal Pain
Weakness
Nausea Weight Loss
Myalgias Vomiting Postural Dizziness
Arthralgias
Headaches
Salt Craving

Signs:
Hypotension
tachycardia
Vitiligo, pigmentation (Primary only)

Lab findings
Hyperkalemia (1o Only) Azotemia
Hyponatremia Anemia
Hypoglycemia Eosinophilia

3

Dx of Primary adrenal insufficiency

Serum Cortisol
less than 3 ug/dl Baseline
less than 20 ug/dl after Cosyntropin (250 ug): ACTH stimulation test and measure serum cortisol (Normal is 20-30 at 30, 60 mins)

Plasma ACTH
greater than 100 pg/ml

Adrenal CT Scan:
small: autoimmune, metabolic
Large: all other causes

*Complete adrenal insufficiency is life threatening

4

Autoimmune Polyglandular syndrome 1 (APS1)

-autoimmune regulator gene assoc
-Adrenal Insufficiency (Addison’s Disease)
Hypoparathyroidism
Type 1 Diabetes Mellitus
Mucocutaneous Candidiasis

5

APS-2

-HLA associated
-Adrenal Insufficiency (Addison’s Disease)
Hypothyroidism (Hashimoto’s Thyroiditis)
Type 1 Diabetes Mellitus

6

Radiology of adrenal insuff. What's the best tool? appearances

CT
small: autimmune, metabolic
large: infection, hemorrhage, metastases

7

Secondary renal insufficiency

low CRF, low ACTH
low cortisol, low adrenal androgens
*normal aldosterone

Causes:
Glucocorticoids (supraphysiologic doses for greater than 3 weeks)
Opioids
Tumor
Surgery
Radiation
Infectious
Hemorrhage
Infiltrative
Metastatic

8

Dx of secondary adrenal insufficiency

Serum Cortisol
less than 3 ug/dl Baseline
less than 20 ug/dl after Cosyntropin (250 ug)

Plasma ACTH
normal or low

Pituitary MRI shows Pathology

9

Tx of Adrenal insuff

Glucocorticoid replacement (Primary + secondary)
Hydrocortisone: 15-25 mg/day
Prednisone: 4-5 mg/day
Dexamethasone: 0.75 mg/day

Mineralocorticoid Replacement (primary only)
Fludrocortisone: .05-0.1 mg/day

10

Primary aldosteronism

-due to hyperplasia or tumor of adrenal gland
-too much aldo produced
-Na retention, excretion of K and H
-Hypertension, hypokalemia, metabolic alkalosis

11

Primary Aldosteronism

Main subtypes:
Aldosterone Producing Adenoma (APA): 34%

Idiopathic Hyperaldosteronism (IHA): 66%

12

Who do we screen for primary aldosteronism?

Hypertensive pts with:
-hypokalemia (spontaneous, provoked with diuretics)
-severe htn (greater than 160/100
-resistant htn (greater than 2 drugs)
-htn onset at less than 20 y
-adrenal incidentaloma

13

Screening tests for primary aldosteronism

morning samle:
plasma aldo
plasma renin activity

Positive screen:
PA greater than 15 ng/dl AND PA/PRA ration greater than 20

Med restriction:
spironolactone

14

Primary aldosteronism confirmation tests

Sodium suppression testing:
measure aldo at beginning and end
-oral salt load (high NaCl diet x 3d)
-IV saline infusion (2L NS over 4 hrs)

These should suppress aldosterone beyond detectable level (establishes dx)

Dx results:
Oral salt load: 24 h urine aldo >12 ug on 3rd day
IV saline infusion PA greater than 10 ng/dl

Med restriction:
spironolactone

15

Primary Aldosteronism surgical results

APA: surgical managment is option
IHA: medical management only

16

Presentation for highly likely APA

age less than 40
severe HTN
severe hypokalemia
plasma aldo greater than 25 ng/dl
urine aldo >30 ug/24 hrs

Surgery?
need abdominal CT
adrenal vein sampling

17

Treatment of APA

Pre-op
aldosterone antag then
Adrenalectomy (laproscopic/open)

Increased aldo, give spironolactone, epleronone

18

Tx IHA

Aldosterone antag plus BP meds (CCB, ACE-I, ARB)

19

Pheochromocytoma

Increased catecholamines:
hypertension, HA, sweating, palpitations
Triad: htn plus HA, sweating, palp

20

Size majority for pheochromocytoma and rule of 10s

greater than 4 cm

Rule of tens:
10% malignant
10% bilateral
10% familial
10% extra-adrenal

21

Pheochromocytoma familial syndromes

MEN type 2A/2B
Von hippel lindau
neurofibromatosis type 1
Familial Paragangliomas (SDH mutations)

-genetic testing avail

22

Who to screen for pheochromocytoma

hypertensive pts with:
-spells (HA, sweating, palp)
-severe HTN (>160/100)
-resistant HTN (>2 drugs)
-adrenal incidentaloma
-familial syndrome

23

Screening tests for pheochromocytoma

-urine metanephrines: positive greater than 1300 ug/24h
-urine catecholamines: positive greater than 2 fold increase
-plasma metanephrines:
positive greater than 0.5 nmol/L
positive normetanephrine: greater than 2 fold increased

BEST: urine metanephrines and catecholamines

24

Pheochromoctyoma false positives

Levodopa
Ethanol
Tricyclic antidepressants (inc. Flexeril)
Buspirone (and other anti-psychotics)
Acetaminophen (plasma metanephrines)
Amphetamines, Opioids
Clonidine withdrawal
Renal failure
Sleep apnea
Physical stress

25

Management of pheochromocytoma

Preop:
Alpha Blockers (1st):
Phenoxybenzamine: 20-100 mg qd
Prazosin: 1-5 mg BID
Terazosin: 2-10 mg QD
Doxazosin: 1-16 mg QD

Beta blockers (2nd after alpha)

CCBs (alone)

Then:
adrenalectomy (laparscopic/open)

Tx effects: volume expansion, vasodilation, rate control

26

Cushing's syndrome

ACTH secreting pituitary tumor (80% of endogenous Cushing's syndrome)

-high ACTH
-high cortisol
-normal aldosterone
-increased adrenal androgens

Ectopic ACTH secreting tumor (10% of endogenous cushing's syndrome)
-high ACTH, cortisol, and adrenal adrogens
-normal aldo

Cortisol secreting adrenal tumor:
10% of endogenous Cushing's syndrome
low ACTH
high cortisol
Normal: aldosterone, adrenal androgens

27

Cushing's Syndrome clinical features

Sx:
Fatigue
Weight Gain
Weakness
Facial Plethora
Headaches
Easy Bruising

Signs
Hypertension Muscle Weakness
Central Obesity
Thin Skin
Purple Stretch-Marks Hirsutism

Lab:
hyperglycemia
hyperlipidemia

28

Screening test for Cushing's

24 hr urine cortisol elevated
bedtime salivary cortisol elevated
1 mg DST (dexamethasone suppresion test): cortisol greater than 1.8 ug/dl

29

DDX of Cushings and ACTH and DST results

Pituitary ACTH secreting tumor:
ACTH normal/high
DST: suppression

Ectopic ACTH secreting tumor:ACTH high or very high
DST: no suppression

Adrenal Cortisol secreting tumor:
ACTH: low
DST no suppression

30

Cushing's syndrome Tx

Surgery: Pituitary, Chest, Abdomen

ACTH Secretion Inhibitors:
Cabergoline, Pasireotide

Cortisol Synthesis Inhibitors
Ketoconazole, Metyrapone, Etomidate

Adrenolytic Agents
Mitotane

Cortisol Receptor Blockers
Mefispristone

31

Adrenal incidentaloma

-frequently found on abdominal imaging
-usually benign:
Most cancers >6 cm
-Usually non-func
-most common hormone abnormality: autonomous cortisol secretion

32

Causes of adrenal incidentaloma

Adrenal Cortical Adenoma
Adrenal Cortical Carcinoma
Pheochromocytoma
Adrenal Metastases
Myelolipoma
Nodular Adrenal Hyperplasia

33

Eval of Adrenal incidentaloma

Determine Cancer Risk
Measure Size and Growth Over Time

Determine if Hormonally Active
Cortisol: 1 mg Overnight Dexamethasone Test
Aldosterone: Plasma Aldosterone and Renin
Androgens: Serum Testosterone and DHEA-S
Catecholamines: Urine Catecholamines and Metanephrines

34

Management of Adrenal incidentaloma

-Surgical removal:
size greater than or equal to 4.5 cm
progressive growth
hormone secretion

Monitor (6 mos, 12 mos, 24 mos)
Size less than 4.5 cm
No hormone secretion