Adrenal Disorders Flashcards by Aubrae Anne

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

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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

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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

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Autoimmune Polyglandular syndrome 1 (APS1)

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

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APS-2

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

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

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

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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

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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

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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

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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

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Primary Aldosteronism

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

Idiopathic Hyperaldosteronism (IHA): 66%

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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

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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

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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

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Primary Aldosteronism surgical results

APA: surgical managment is option
IHA: medical management only

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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

Treatment of APA

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

Increased aldo, give spironolactone, epleronone

Tx IHA

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

Pheochromocytoma

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

Size majority for pheochromocytoma and rule of 10s

greater than 4 cm

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

Pheochromocytoma familial syndromes

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

-genetic testing avail

Who to screen for pheochromocytoma

hypertensive pts with:

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

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

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

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

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 ```

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

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

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

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

Adrenal incidentaloma

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

Causes of adrenal incidentaloma

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

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

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