Pituitary/Adrenal Glands

Question 1

Enzyme converts to NE to epi?

Answer 1

PNMT, found only in adrenal medulla

Question 2

Pheochromocytoma 10% rule

Answer 2

10% are: malignant, bilateral, in children, part of MEN, extra-adrenal (organ of Zuckerkandl at Ao bifurcation = most common)

Question 3

Preop tx of pheochromocytoma

Answer 3

alpha block first, then beta block if tachycardic

Question 4

Nelson syndrome

Answer 4

post adrenalectomy (10%), incr ACTH, pigmentation, vision changes from incr pituitary response

Question 5

Waterhouse Friedrickson syndrome

Answer 5

adrenal hemorrhage a/w meningococcal sepsis

Question 6

Conn’s syndrome

Answer 6

hyperaldosteronism = 80% adenoma, 20% bilateral hyperplasia (see with postural stimulation test). HTN, low K, high Na

Question 7

Addison’s disease

Answer 7

low aldosterone and glucocorticoids = low Na, high K, hypoglycemia. Crisis presents similar to sepsis with hypoTN, fever; steroids are diagnostic and therapeutic ADDison’s = ADrenals Down

Question 8

Congenital Adrenal Hyperplasia

Answer 8

21-hydroxylase deficiency = most common

Question 9

Classic vision change with pituitary mass affect

Answer 9

Bitemporal hemianopsia

Question 10

Chromophobe pituitary adenoma

Answer 10

non-functional, see decr GH, FSH, LH, TSH, ACTH

Question 11

Sheehan syndrome

Answer 11

postpartum lack of lactation, persistent amenorrhea

Question 12

Adrenal venous drainage left vs right?

Answer 12

Left: adrenal vein –> left renal vein Right: adrenal vein –> IVC

Question 13

Three zones of adrenal cortex?

Answer 13

Glomerulosa: aldosterone Fasciculata: glucorticoids Reticularis: androgens

Question 14

Adrenal medulla origin and produces what?

Answer 14

Neural crest Catecholamines: NE, epi, dopamine

Question 15

Actions of angiotensin 2

Answer 15

Vasoconstriction Release of aldosterone: Na retention, K excretion

Question 16

Primary hyperaldosteronism (Conn’s syndrome) sx, dx and tx

Answer 16

Sx: HTN, hypokalemia Dx: CT adrenal protocol, serum aldosterone:renin ratio is >30:1 Tx: >1 cm macroadenoma: lap adrenalectomy <1 cm: adrenal venous sampling hyperplasia: manage medically

Question 17

Cushing syndrome sx

Answer 17

buffalo hump moon face central obesity

Question 18

Cushing syndrome causes

Answer 18

ACTH secreting pituitary tumor Ectopic ACTH secreting tumor (small cell lung cancer) Adenomas: Cortisol producing adrenal tumors

Question 19

Cushing syndrome dx

Answer 19

screening - 1 mg dex suppression test confirm - serum ACTH and DHEA levels low

Question 20

Causes of primary adrenal insufficiency

Answer 20

MCC US: autoimmune MCC Worldwide: TB

Question 21

Symptoms of adrenal insufficiency

Answer 21

Fatigue, anorexia, abd pain, skin hyperpigmentation

Question 22

Addisonian crisis

Answer 22

Refractory shock CV collapse Dx: stim test Tx: treat empirically if high suspicion, can give dex so it does not interfere with stim test

Question 23

When to tx an adrenal incidentaloma?

Answer 23

<4 cm with benign characteristic on CT scan: observe, repeat imaging in 6 months Functional, >6 cm, worrisome imaging: adrenalectomy 4-6 cm: individualized treatment Myeolipoma or cyst: does not need to be resected unless symptomatic

Question 24

Adrenocortical carcinoma tx

Answer 24

Open adrenalectomy to avoid tumor spillage 60% are hyperfunctioning

Question 25

Pheochromocytoma dx

Answer 25

Screening: plasma metanephrine Confirmatory: 24hr urine metanephrine

Question 26

Pheochromocytoma tx

Answer 26

alpha-blockade (phenoxybenzamine) -\> beta-blockade -\> resect unopposed alpha stimulation = dangerously high blood pressures lap adrenalectomy or RP approach

Question 27

MC site for extra-adrenal pheochromocytoma?

Answer 27

Organ of Zuckercandl - aortic bifurcation

Question 28

What does alpha-1 receptor mediate?

Answer 28

Vasoconstriction Pupillary dilation Intestinal relaxation Uterine contraction

Question 29

What does alpha-2 receptor mediate?

Answer 29

Vasoconstriction Feedback inhibition of NE release from sympathetic neurons Inhibits renin release and insulin release

Question 30

What does beta-1 receptor mediate?

Answer 30

Increases force and rate of cardiac muscle contraction Increases lipolysis Increases amylase production

Question 31

What does beta-2 receptor mediate?

Answer 31

Relaxes smooth muscle Increases glycogenolysis Increases insulin and glucagon secretion Increases renin secretion

Question 32

Which familial syndromes are a/w pheochromocytoma?

Answer 32

Isolated familial pheochromocytoma MENIIa MENIIb Von Recklinghausen neurofibromatosis

Question 33

Classic post-op complications after pheochromocytoma removal?

Answer 33

Persistent HTN Hypotension Hypoglycemia - decreased circulating catecholamines, increased insulin release Bronchospasm - decreased beta-2 activation

Question 34

Recurrence rate of pheo after resection?

Answer 34

5-10% Monitor catecholamine levels annually for the first 5 years

Question 35

What percentage of patients with VHL syndrome have a pheo?

Answer 35

50%

Question 36

What should be done prophylactically in patients with known adrenal insufficiency undergoing surgery?

Answer 36

Stress dose steroids 100 mg cortisol IV on the day of surgery and then every 8hr Taper as tolerated post-op to maintenance dose

Question 37

MEN Syndromes

Answer 37

Diamond, square, triangle

Question 38

MEN I, IIA, IIB: 1. Chromosome 2. Inheritance pattern 3. Medullary thyroid cancer? 4. MTC course 5. Parathyroid disease? 6. Pheo? 7. Phenotype 8.

Answer 38