Mcphs Cushing's Syndrome/disease And Aldosteronism

Front 1

This is caused by prolonged exposure to excessive glucocorticoids?

Back 1

Cushing's Syndrome

Front 2

What are six things seen in the presentation of Cushing's syndrome?

Back 2

1. Obesity

2. Metabolic complications

3. Dermatologic manifestations

4. Reproductive changes

5. Musculoskeletal manifestations

6. Neuropsychiatric changes

Front 3

What is specifically seen with the obesity associated with Cushing's syndrome?

Back 3

It is progressive central obesity, dorsocervical hump, moon face, and supraclavicular fat pads

Front 4

What metabolic complications can occur with Cushing's syndrome?

Back 4

Glucose intolerance and hypertension

Front 5

What dermatologic manifestations occur with Cushing's syndrome?

Back 5

Skin atrophy, fragility, bruisability, purple striae, hyperpigmentation (if ACTH is high), and fungal infections.

Front 6

If you see purple striae or hyperpigmentations in Cushing's syndrome that can mean what?

Back 6

That ACTH is high

Front 7

What reproductive changes are seen with Cushing's syndrome?

Back 7

Menstrual irregularities, hirsutism, acne, and other signs of virilization.

Front 8

What musculoskeletal manifestations are seen with Cushing's syndrome?

Back 8

Proximal myopathy, muscle wasting, and osteoporosis

Front 9

What neuropsychiatric changes are seen with Cushing's syndrome?

Back 9

Labile mood, agitated depression, panic attacks, mild paranoia, insomnia

Front 10

What is the most common cause of Cushing's syndrome?

Back 10

Exogenous glucocorticoids - excess glucocorticoids

Front 11

When there are excess glucocorticoids coming from an endogenous source, what can those sources be?

Back 11

1. Adrenal adenoma
2. Carcinoma
3. Hyperplasia

Front 12

What is responsible for 80% of endogenous cases of Cushing's syndrome?

Back 12

Excess ACTH

Front 13

Cushing's disease, is the most common endogenous cause of Cushing's syndrome. What is the primary cause of it and what are the two other causes?

Back 13

ACTH secreting pituitary adenoma is the primary cause.

The two other causes for endogenous Cushing's syndrome are ectopic ACTH, ectopic CRH

Front 14

What are the three steps to laboratory diagnosis of Cushing's Syndrome?

Back 14

1. Documenting the presence of hypercortisolism.

2. Determining if cortisol excess is from excess ACTH or not.

3. Determining the source of ACTH if the cortisol excess is from excess ACTH

Front 15

What imaging is done to diagnose Cushing's?

Back 15

CT, MRI, Octreotide Scan

Front 16

When you are documenting the presence of hypercortisolism to make your diagnosis of Cushing's, what is the best screening test to use?

Back 16

The 24 hour urine free cortisol is the best screening test - elevated 3X normal on 2 occasions solidifies confirmation.

Front 17

What are three other methods, apart from 24 hour urine free cortisol testing, to document the presence of hypercortisolism?

Back 17

1-mg overnight dexamethasone suppression test - high rate of false positives

Late evening salivary cortisol measurement

Midnight plasma cortisol measurement

Front 18

When determining if cortisol excess is from excess ACTH (ACTH dependent) or not (ACTH independent), what test is performed?

Back 18

Plasma ACTH assay

Front 19

How do you interpret the results of the Plasma ACTH assay?

1. if low, then cortisol excess is?

2. if high, then cortisol excess is?

If still unsure after this test then may check?

Back 19

1. ACTH independent

2. ACTH dependent

3. If still unsure, then may check a CRH stimulation test, if blunted response, then ACTH independent

Front 20

When determining the source of the ACTH, if the cortisol excess is ACTH dependent, How do you figure out if the ACTH production is ectopic or not?

Back 20

ACTH in Cushing's disease is usually suppressible (by dexamethasone), and stimulatable (CRH), while ectopic ACTH is not suppressible or stimulatable.

Front 21

What tests are used to determine the source of ACTH if cortisol excess is determined to be ACTH dependent?

Back 21

High dose dexamethasone suppression test

Front 22

If you do a high dose dexamethasone test, how do you determine what the source of ACTH is if cortisol excess is determined to be ACTH dependent?

Back 22

If still unclear after high dose dexamethasone suppression test, then perform a inferior petrosal sinus sampling to look for central to peripheral gradient (Cushing's disease) If there is no gradient then must search for ectopic ACTH

Front 23

How do you treat exogenous cushing's syndrome?

Back 23

Gradual withdrawal of glucocorticoid (so as to avoid precipitation of adrenal insufficiency).

Front 24

How do you treat ACTH-independent Cushing's syndrome?

Back 24

Unilateral adrenalectomy for adenoma or carcinoma

Bilateral adrenalectomy for bilateral hyperplasia

Replacement glucocorticoids +/- mineral corticoids

Front 25

How do you treat Cushing's disease?

Back 25

Transphenoidal microadenectomy is the tx of choice

In other cases, subtotal resection of anterior pituitary

Incomplete resection may be followed with repeat surgery or pituitary irradiation

Front 26

How do you treat Ectopic ACTH and CRH syndrome?

Back 26

Surgical resection if possible

Medical treatment for malignancy

Front 27

With this Cushing patients should be monitored with plasma cortisol and 24 hour urinary cortisol to keep normal range.

Ketoconazole is usually first line

Back 27

Medical therapy with adrenal enzyme inhibitors

Front 28

In cushings, what are the other adrenal enzyme inhibitors besides the first line TX ketoconazole?

Back 28

Mitotane

Metyrapone

Aminoglutethamide

Front 29

This is a non-suppressible hypersecretion of aldosterone?

Back 29

Primary hyperaldosteronism

Renin-independent (ie, renin is low)

Front 30

What two findings should trigger thoughts of hyperaldosteronism?

Back 30

Hypertension and Hypokalemia

Front 31

This accounts for about 2/3 of hyperaldosteronism cases. Usually due to bilateraly adrenal hyperplasia.

Back 31

Idiopathic hyperaldosteronism

Front 32

These usually account for a 1/3 of cases of hyperaldosteronism?

Back 32

Aldosterone producing adenomas

Front 33

Apart from aldosterone producing adenomas and bilateral adrenal hyperplasia, what are four other causes of hyperaldosteronism?

Back 33

1. Unilateral primary adrenal hyperplasia

2. Aldosterone-producing adrenocortical carcinomas

3. Aldosterone producing ovarian tumor

4. Familial hyperaldosteronism

Front 34

What are the seven signs that present with hyperaldosteronism?

Back 34

1. Hypertension - associated with more end organ damage and greater cardiovascular risk than essential HTN

2. Weakness, muscle cramps
3. Headache, fatigue, palpitations, polyuria
4. Mild hypernatremia
5. Hypokalemia
6. Metabolic alkalosis
7. Hypomagnesemia

Front 35

How do you screen for hyperaldosteronism?

Back 35

PAC/PRA ratio

Front 36

How do you confirm diagnosis of hyperaldosteronism?

Back 36

Sodium loading

Front 37

How do you localize the cause of hyperaldosteronism?

Back 37

1. CT or MRI
2. Adrenal venous sampling

Front 38

This is the ration of plasma aldosterone concentration to plasma renin activity. It is the screening test of choice for hyperaldosteronism.

Back 38

PAC/PRA ratio

Front 39

What will interfere with PAC/PRA ratio testing?

Back 39

Aldosterone receptor blockers, ACE-inhibitors, ARB's interfere with test

Front 40

What are the indications for screening for hyperaldosteronism?

Back 40

1. Hypokalemia
2. Treatment-resistant hypertension (3 drugs)
3. Severe HTN
4. Early-onset HTN
5. Adrenal mass
6. Family history of hyperaldosteronism

Front 41

What is confirmation of a hyperaldosteronism diagnosis?

Back 41

Nonsuppression of aldosterone during sodium loading (PO or IV)

Front 42

A urine aldosterone >14mcg/24 hours confirms?

Back 42

Nonsuppresibility of aldosterone, which means the patient is dealing with hyperaldosteronism

Front 43

A urine sodium of >200 mEq/24 hours confirms?

Back 43

Adequate sodium loading

Front 44

With hyperaldosteronism, when imaging with CT or MRI and you see a large mass with atypical features this makes what more likely?

Back 44

Carcinoma

Front 45

When performing adrenal venous sampling if aldosterone is much higher (4X) in on adrenal vein, then this is consistent with?

If aldosterone is equal between sides, then this is consistent with?

Back 45

1. Adenoma

2. Hyperplasia

Front 46

This is the treatment of choice for hyperaldosteronism caused by unilateral primary adrenal hyperplasia and for aldosterone producing adenomas?

Back 46

Unilateral Laparoscopic Adrenalectomy

Front 47

This is reserved for patients, suffering from hyperaldosteronism, with bilateral disease or patients with unilateral disease who refuse surgery or are poor surgical candidates.

Back 47

Medical management of hyperaldosteronism

Front 48

What are the effective agents when managing hyperaldosteronism medically?

Back 48

Spironolactone
Eplerenone
Amiloride
Triamterene

Front 49

With this renin output is high. All of the following are causes for this?

Hyperkalemia
Hyponatremia
Hypotension
Decreased effective circulating volume - CHF, cirrhosis, nephrotic syndrome

Back 49

Secondary hyperaldosteronism

Front 50

Hypoaldosteronism (Type IV RTA) is caused by?

Back 50

mineralcorticoid deficiency

Front 51

Hypoaldosteronism (Type IV RTA) presents as?

Back 51

Hyperkalemia, Hyperchloremic metabolic acidosis

Front 52

How do you treat hypoaldosteronism (Type IV RTA)?

Back 52

Fludrocortisone

Front 53

What are the two etiologies of hypoaldosteronism?

Back 53

1. Aldosterone deficiency
2. Decreased response to aldosterone

Front 54

With hypoaldosteronism caused by aldosterone deficiency, what three things are associated factors?

Back 54

1. Hyporeninemia - Due to kidney damage, as from diabetes mellitus, drugs, or other forms of kidney disease.

2. ACE inhibitors

3. Adrenal insufficiency

Front 55

With hypoaldosteronism caused by a decreased response to aldosterone, what two things are associated factors?

Back 55

1. Drugs

2. Defective aldosterone receptor - Pseudohypoaldosteronism