Section 4: Adrenal Disorders

Question 1

Clinical presentation of Cushing syndrome

Answer 1

• Fat redistribution: Truncal obesity, “moon face,” buffalo hump, thin arms and legs
• Easy bruising and striae: Loss of collagen from the cortisol thins the skin
• Hypertension: From fluid and sodium retention (look for hypokalemia in hyperaldosteronism)
• Muscle wasting
• Hirsutism: From increased adrenal androgen levels

Question 2

Anosmia with hypogonadism (low GnRH, FSH, and LH)

Answer 2

Kallman’s syndrome

Question 3

Clinical features of Klinefelter’s syndrome

Answer 3

• Insensitivity of the FSH and LH receptors on their testicles XXY on karyotype
• The FSH and LH levels are very high, but no testosterone is produced from the testicles.

Question 4

Features common to all forms of congenital adrenal hyperplasia (CAH)

Answer 4

• Elevated ACTH
• Low aldosterone and cortisol levels
• Treatable with prednisone, which inhibits the pituitary.

Question 5

What forms of CAH has HTN

Answer 5

11 and 17 hydroxylase deficiencies

Question 6

Virilization is seen in which of the CAH

Answer 6

21 and 11 hydroxylase deficiencies

Question 7

Diagnosis: CAH with increased 17 hydroxyprogesterone level

Answer 7

21 hydroxylase deficiency

Question 8

Cause of Hirsutism in 21 and 11 hydroxylase deficiencies

Answer 8

Increased andrenal androgens

Question 9

List the steps in the diagnosis of hypercortisolism (Cushing syndrome)

Answer 9

1. Establish the presence of hypercotisolism
2. Establish the cause of hypercotisolism

Fischer, Conrad (2012-11-01). Master the Boards USMLE Step 2 CK (Page 119). Kaplan Medical Test Prep. Kindle Edition.

Question 10

What is the best initial test(s) to establish the presence of hypercortisolism?

Answer 10

1. 24-hour urine cortisol
2. 1 mg overnight dexamethasone suppression test

Fischer, Conrad (2012-11-01). Master the Boards USMLE Step 2 CK (Page 119). Kaplan Medical Test Prep. Kindle Edition.

Question 11

The 1 mg overnight dexamethasone suppression test should normally suppress the morning cortisol level. If this suppression occurs, what does this imply?

Answer 11

Hypercortisolism is excluded

Fischer, Conrad (2012-11-01). Master the Boards USMLE Step 2 CK (Page 119). Kaplan Medical Test Prep. Kindle Edition.

Question 12

List the causes of false positive 1 mg overnight suppression testing

Answer 12

• Depression
• Alcoholism
• Obesity

Question 13

Hypercotisolism + Decreased ACTH level

What is the source of the hypercotisolism?

Answer 13

Adrenal source

Question 14

1. List the best initial test to determine the cause (source) or location of hypercotisolism
2. What is the interpretation of the possible results of (1)?

Answer 14

1. Adrenocorticorticotropic hormone (ACTH) testing
2. Low ACTH means an adrenal source while a high ACTH means either a pituitary source or an ectopic source (e.g. lung cancer, carcinoid)

Question 15

Hypercortisolism is established and there is elevated ACTH level, what is the next best test to do? Interpret the results of the test

Answer 15

The next best test to do is the dexamethasone suppression test

If the dexamethasone suppression test suppresses ACTH then the source is the pituitary

If the dexamethasone suppression test does not suppresses ACTH then the source is an ectopic one such as lung cancer or carcinoid

Question 16

1. Hypercortisolism is established and there is elevated ACTH level and dexamethasone suppression test suppresses ACTH, what is the next best test to do?
2. What will be the next line of action based on the result of (1)?

Answer 16

1. MRI of the brain
2. If the MRI shows a pituitary lesion then treat. If the MRI does not show a clear
   pituitary lesion, sample the inferior petrosal sinus for ACTH, possibly afterstimulating the patient with corticotrophin releasing hormone (CRH). An elevated ACTH from the venous drainage of the pituitary confirms the pituitary as the source. The petrosal venous sinus must be sampled because somepituitary lesions are too small to be detected on MRI.

Question 17

1. Hypercortisolism is established and there is elevated ACTH level, and dexamethasone suppression test does not suppress ACTH, what is the next best test to do?

Answer 17

Scan the chest for lung cancer or carcinoid

Question 18

Cortisol is a stress hormone that is an antiinsulin. In addition, there is some aldosteronelike effect of cortisol that has an effect on the kidney’s distal tubule of excreting potassium and hydrogen ions.

List the metabolic effects of hypercortisolism

Answer 18

• Hyperglycemia
• Hyperlipidemia
• Hypokalemia
• Metabolic alkalosis

Question 19

What is the effect of hypercortisolism on leukocytes?

Answer 19

Leukocytosis from demargination of white blood cells. At least half of
white cells in the blood are on the vessel wall waiting for an acute stress to
come into circulation. They are like parked police cars waiting to be called

Fischer, Conrad (2012-11-01). Master the Boards USMLE Step 2 CK (Page 120). Kaplan Medical Test Prep. Kindle Edition.

Question 20

Step to take in the evaluation of adrenal “Incidentaloma” (an unexpected, asymptomatic adrenal lesion found on CT)?

Answer 20

• Metanephrines of blood or urine to exclude pheochromocytoma
• Renin and aldosterone levels to exclude hyperaldosteronism
• 1 mg overnight dexamethasone suppression test to exclude hypercortisolism

Question 21

Fill in the blanks for the tests to be done for hypercorticolism

Answer 21

Results are shown below