Adrenal Disorders Flashcards

1
Q

Primary Adrenal Insufficiency

A

-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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2
Q

Clinical Features of Adrenal insuff

A
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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3
Q

Dx of Primary adrenal insufficiency

A

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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4
Q

Autoimmune Polyglandular syndrome 1 (APS1)

A

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

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5
Q

APS-2

A

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

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6
Q

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

A

CT

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

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7
Q

Secondary renal insufficiency

A

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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8
Q

Dx of secondary adrenal insufficiency

A

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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9
Q

Tx of Adrenal insuff

A

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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10
Q

Primary aldosteronism

A
  • 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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11
Q

Primary Aldosteronism

A

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

Idiopathic Hyperaldosteronism (IHA): 66%

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12
Q

Who do we screen for primary aldosteronism?

A

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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13
Q

Screening tests for primary aldosteronism

A

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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14
Q

Primary aldosteronism confirmation tests

A

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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15
Q

Primary Aldosteronism surgical results

A

APA: surgical managment is option
IHA: medical management only

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16
Q

Presentation for highly likely APA

A
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
Q

Treatment of APA

A

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

Increased aldo, give spironolactone, epleronone

18
Q

Tx IHA

A

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

19
Q

Pheochromocytoma

A

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

20
Q

Size majority for pheochromocytoma and rule of 10s

A

greater than 4 cm

Rule of tens:
10% malignant
10% bilateral
10% familial
10% extra-adrenal
21
Q

Pheochromocytoma familial syndromes

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

-genetic testing avail

22
Q

Who to screen for pheochromocytoma

A

hypertensive pts with:

  • spells (HA, sweating, palp)
  • severe HTN (>160/100)
  • resistant HTN (>2 drugs)
  • adrenal incidentaloma
  • familial syndrome
23
Q

Screening tests for pheochromocytoma

A

-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
Q

Pheochromoctyoma false positives

A
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
Q

Management of pheochromocytoma

A
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
Q

Cushing’s syndrome

A

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
Q

Cushing’s Syndrome clinical features

A
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
Q

Screening test for Cushing’s

A

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

29
Q

DDX of Cushings and ACTH and DST results

A

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
Q

Cushing’s syndrome Tx

A

Surgery: Pituitary, Chest, Abdomen

ACTH Secretion Inhibitors:
Cabergoline, Pasireotide

Cortisol Synthesis Inhibitors
Ketoconazole, Metyrapone, Etomidate

Adrenolytic Agents
Mitotane

Cortisol Receptor Blockers
Mefispristone

31
Q

Adrenal incidentaloma

A

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

32
Q

Causes of adrenal incidentaloma

A
Adrenal Cortical Adenoma
Adrenal Cortical Carcinoma
Pheochromocytoma
Adrenal Metastases
Myelolipoma
Nodular Adrenal Hyperplasia
33
Q

Eval of Adrenal incidentaloma

A

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
Q

Management of Adrenal incidentaloma

A

-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