Pathology II

Question 1

What is the pathophysiology associated with secondary hyperaldosteronism?

Answer 1

Renal perception of low intravascular volume. This results in an overactive renin-angiotensin system (p.296)

Question 2

Name five causes of secondary hyperaldosteronism.

Answer 2

Renal artery stenosis, chronic renal failure, CHF, cirrhosis, nephrotic syndrome (p.296)

Question 3

What lab finding is characteristic of secondary hyperaldosteronism?

Answer 3

Elevated plasma renin (p.296)

Question 4

What is the treatment for secondary hyperaldosteronism?

Answer 4

Spironolactone (p.296)

Question 5

What is Addison’s disease?

Answer 5

Chronic primary adrenal insufficiency due to adrenal atrophy or destruction by disease (p.297)

Question 6

Name three types of disease states that can cause chronic primary adrenal insufficiency.

Answer 6

Autoimmune, Infection (TB), metastasis (p.297)

Question 7

What endocrine hormones are deficient in primary adrenal insufficiency?

Answer 7

Aldosterone and cortisol (p.297)

Question 8

What are the typical clinical findings in a patient with primary adrenal insufficiency?

Answer 8

Hypotension (hyponatremic volume contraction due to lack of aldosterone), hyperkalemia, acidosis, skin hyperpigmentation (due to MSH, a by-product of increased ACTH production from POMC) (p.297)

Question 9

What endocrine hormone is elevated in primary adrenal insufficiency?

Answer 9

ACTH (p.297)

Question 10

What characterizes primary adrenal insufficiency?

Answer 10

Adrenal atrophy and absence of hormone production from all three cortical divisions. Medulla is spared (p.297)

Question 11

What lab finding distinguishes primary adrenal insufficiency from secondary adrenal insufficiency?

Answer 11

High ACTH in primary adrenal insufficiency; decreased pituitary ACTH production in secondary adrenal insufficiency (p.297)

Question 12

What clinical features distinguish primary adrenal insufficiency from secondary adrenal insufficiency?

Answer 12

Secondary adrenal insufficiency has no hyperpigmentation and no hyperkalemia (p.297)

Question 13

What is Waterhouse- Friderichsen syndrome?

Answer 13

Acute primary adrenal insufficiency due to adrenal hemorrhage (p.297)

Question 14

What four pathologies are associated with Waterhouse Friderichsen syndrome?

Answer 14

Neisseria meningitidis, septicemia, DIC, and endotoxic shock(p.297)

Question 15

What is a pheochromocytoma?

Answer 15

The most common tumor of the adrenal medulla in adults. Symptoms occur in spells and are relapsing/remitting (p.297)

Question 16

From what cells are pheochromocytomas derived?

Answer 16

Chromaffin cells (which arise from neural crest) (p.297)

Question 17

Where do chromaffin cells arise from?

Answer 17

Neural crest (p.297)

Question 18

What is most commonly secreted by pheochromocytomas?

Answer 18

Epinephrine, Norepinephrine, dopamine (p.297)

Question 19

What symptoms are associated with pheochromocytomas?

Answer 19

Episodic hyperadrenergic symptoms- pressuire (elevated BP), Pain (headache), Perspiration, Palpitations (tachycardia), Pallor (p.297)

Question 20

What lab findings are consistent with a diagnosis of pheochromocytoma?

Answer 20

Urinary VMA and plasma catecholamines are elevated (p.297)

Question 21

What is Vanillylmandelic acid?

Answer 21

A breakdown product of norepinephrine and epinephrine. Elevated in the urine with pheochromocytoma (p.297)

Question 22

What three conditions are pheochromocytomas associated with?

Answer 22

MEN 2A, MEN 2B, Neurofibromatosis type 1 (p.297)

Question 23

What is the treatment for pheochromocytoma?

Answer 23

Surgical removal after effective a-and b-blockade is achieved (p.297)

Question 24

What treatment is required before a patient can undergo surgery to remove a pheochromocytoma?

Answer 24

Before surgery, irreversible a-antagonists (phenoxybenzamine) must be given to avoid a hypertensive crisis. B-blockers are then given to slow heart rate (p.297)

Question 25

What is the rule of 10s associated with pheochromocytoma?

Answer 25

10% are: malignant, bilateral, extra-adrenal, calcified, in children (p.297)

Question 26

Outline the pathway of epinephrine production.

Answer 26

Phenylalanine –> Tyrosine –> L-Dopa –> Dopamine –> Norepinephrine –> Epinephrine (p.297)