Mcphs Chronic Adrenal Insufficiency, Adrenal Crisis, Pheochromocytoma

Front 1

This is primary chronic adrenal insufficiency. It is a problem at the level of the adrenal glands. Autoimmune is the most common cause in the USA, infections such as TB in other countries are main cause.

Back 1

Addison's Disease

Front 2

With this you see Hemorrhagic infarction, metastatic disease, drugs, severe inflammatory disease, and inherited conditions.

May have symptoms and signs of glucocorticoid, mineralcorticoid, and in women-androgen deficiency

Causes skin pigmentation that can be subtle or strikingly dark

Volume and sodium depletion and potassium excess

Back 2

Addison's Disease

Front 3

This is when you have chronic adrenal insufficiency and the problem is at the pituitary.

Panhypopituitarism, isolated ACTH deficiency, megestrol, opiates, abrupt cessation of high dose glucocorticoids, or correction of Cushing's. Usually have normal mineralcorticoid function, so hyperkalemia is not present. ACTH is not elevated and there is no skin pigmentary changes.

Back 3

Secondary chronic adrenal insufficiency.

Front 4

This is when you have chronic adrenal insufficiency and the problem is at the hypothalamus.

Panhypopituitarism, isolated ACTH deficiency, megestrol, opiates, abrupt cessation of high dose glucocorticoids, or correction of Cushing's. Usually have normal mineralcorticoid function, so hyperkalemia is not present. ACTH is not elevated and there is no skin pigmentary changes.

Back 4

Tertiary chronic adrenal insufficiency

Front 5

With this, most patients have generalized weakness, fatigability, anorexia and weight loss.

GI Sx (Abd pain, N/V/D/C)

Hypotension

Electrolyte Abnormalities - Hyponatremia - due to combination of aldosterone deficiency as well as increased ADH release

Hyperkalemia - due to aldosterone deficiency

Back 5

Primary chronic adrenal insufficiency - Addison's Disease

Front 6

What are the 6 manifestations of primary chronic adrenal insufficiency?

Back 6

1. Hyperpigmentation
2. Vitilligo
3. Sexual dysfunction
4. Musculoskeletal symptoms
5. Articular cartilage calcification
6. Psychiatric manifestations

Front 7

With these, weakness, fatigability, myalgia, arthralgia, and psychiatric symptoms can all occur due to glucocorticoid deficiency.

Hyperpigmentation is not present, no dehydration, and hypotension is less prominent.

Hyponatremia may be present due to ADH

Hyperkalemia is not present

Gastrointestinal symptoms are less common

Hypoglycemia is more common

Back 7

Secondary and Tertiary chronic adrenal insufficiency

Front 8

What is the three stage process for diagnosis of adrenal insufficiency?

Back 8

1. Demonstrate inappropriately low cortisol secretion.

2. Determine whether cortisol deficiency is dependent or independent of ACTH deficiency, and evaluate mineralcorticoid secretion in patients without ACTH deficiency

3. Seek a treatable cause of primary adrenal insufficiency

Front 9

Low AM serum or salivary cortisol is evidence of?

Back 9

Adrenal insufficiency

Front 10

A subnormal response to this test establishes the diagnosing of adrenal insufficiency. A normal response to the high dose test excludes primary disease, but does not completely exclude secondary.

Back 10

Short ACTM (cosyntropin) stimulation test

Front 11

What therapeutic interferes with ACTH testing, and what doesn't?

Back 11

1. Hydrocortisone interferes

2. Dexamethasone doesn't interfere

Front 12

What is the plasma basal ACTH concentration in primary adrenal insufficiency and secondary/tertiary adrenal insufficiency?

Back 12

1. Primary - High

2. Secondary/Tertiary - Low or low/normal

Front 13

In this test, the secondary or tertiary disease states may see recovery of the adrenal function, but not in the primary disease state.

Back 13

Long ACTH stimulation test

Front 14

This test is used to differentiate between secondary and tertiary chronic adrenal insufficiency. There is little or no response in secondary and an exaggerated response in tertiary.

Back 14

CRH stimulation test

Front 15

An abdominal CT will show what in Autoimmune based Addison's disease?

Back 15

small noncalcified adrenal glands

Front 16

If you can't clearly diagnose Addison's as being autoimmune based off of the CT, what should be done?

Back 16

A chest X-Ray to look for TB, fungal infection, or cancer - in which case the adrenals will be enlarged.

Front 17

What are the TX options for adrenal insufficiency?

Back 17

Glucocorticoid replacement
Mineralcorticoid replacement
Androgen replacement

Front 18

What is the drug of choice for corticosteroid replacement in chronic adrenal insufficiency?

Back 18

Hydrocortisone

Front 19

All forms of stress (infection, trauma, surgery) require what?

Back 19

an increase in dose of hydrocortisone in proportion to severity of stress

Front 20

What are the four things needed to educate patients about with chronic adrenal insufficiency?

Back 20

1. Emergency precautions - medic alert bracelet

2. Patient education

3. Treatment of "minor stress" - increase glucocorticoid to 3X dose for 3 days

4. Emergency TX for "severe stress" - Inject contents of pre-filled dexamethasone syringe and get to provider ASAP

Front 21

This is an acute adrenal insufficiency. It is a medical emergency. May occur in the course of treatment of chronic insufficiency or may be the presenting manifestation of adrenal insufficiency. More commonly seen in Addison disease than in secondary or tertiary adrenal insufficiency.

Back 21

Adrenal crisis

Front 22

What are the Precipitants of Adrenal Crisis?

Back 22

1. Previously undiagnosed adrenal insufficiency subjected to stress.

2. Previously diagnosed adrenal insufficiency failing to take stress doses.

3. Bilateral adrenal infarction or hemorrhage

4. Pituitary apoplexy

5. Sudden glucocorticoid withdrawal (PO or INH)

6. Giving thyroxine to patient w/hypoadrenalism

7. Etomidate

Front 23

Adrenal crisis most commonly presents as?

Back 23

Shock

Front 24

Apart from seeing shock with adrenal crisis, what are two other manifestations?

Back 24

Abdominal tenderness

Fever

Front 25

What are the steps to manage adrenal crisis?

Back 25

1. Establish IV

2. Draw stat electrolytes and glucose, and measure plasma cortisol and ACTH, don't wait for results

3. Give fluids (isotonic) as quickly as tolerated.

4. Inject 4mg of dexamethasone IV

Front 26

This is a tumor arising from catecholamine producing chrommafin cells in the adrenal medulla.

Back 26

Pheochromocytoma

Front 27

What is the classic triad of pheochromocytoma?

Back 27

Headache, sweating and palpitations

Front 28

What is the most common physical exam finding with pheochromocytoma?

Back 28

Hypertension

Front 29

The paroxysms of pheochromocytoma may be precipitated by ?

Back 29

displacement of abdominal contents. Typically lasting 30-40 minutes and tend to increase in frequency and severity over time.

Front 30

What are the 2 essential components for diagnosis of pheochromocytoma?

Back 30

Biochemical confirmation

Anatomic localization

Front 31

Initial testing for pheochromocytoma should include measurement of?

Back 31

Fractionated metanephrines in plasma or urine - normametanephrine and metanephrine.

Front 32

What are two tests that are occasionally helpful in diagnosing pheochromocytoma?

Back 32

Clonidine suppression test and glucagon stimulation test.

Front 33

What are four factors that interfere with biochemical testing in pheochromocytoma?

Back 33

1. stimulation of endogenous catecholamines.

2. Exogenous catecholamines

3. Drugs that alter catecholamine metabolism

4. Drugs that interfere with biochemical assays

Front 34

What do you do in pheochromocytoma to get anatomic localization?

Back 34

Abdominal CT or MRI

Front 35

If the CT or MRI are negative for pheochromocytoma, despite strong clinical and biochemical evidence, then what do you do?

Back 35

Nuclear medicine scan

Front 36

What is the TX for pheochromocytoma?

Back 36

Surgical resection is the definitive therapy.

Front 37

What is the preferred drug for pre-op control of blood pressure?

Back 37

Phenoxybenzamine - an alpha blocker

Front 38

These should not be started until adequate alpha blockade has been achieved or could precipitate hypertensive crisis.

Back 38

Beta blockers.