adrenal 1

Question 1

the medulla is composed of post ganglionic sympathetic ____ cells

Answer 1

chromaffin cells

Question 2

the medulla releases ____ into the blood

Answer 2

catecholamines

Question 3

cortex is derived from embryonic ____

Answer 3

mesoderm.

Question 4

medulla is derived from embryonic _____

Answer 4

neural crest cells

Question 5

ACTH is derived from _____

Answer 5

POMC - Proopiomelanocortin

Question 6

signal transduction pathway activated by ACTH

Answer 6

cAMP/PKA

Question 7

acute stimulation of ACTH

Answer 7

• activation of steroidogenic enzymes

Question 8

ACTH stimulates uptake of______ into cells of the adrenal cortex and activation of several downstream enzymes

Answer 8

cholesterol

Question 9

aldosterone synthase is primarily regulated by

Answer 9

mainly by angiotensin II, not ACTH!

Question 10

effects of excess cortisol on carbohydrate and lipid metabolism

Answer 10

• increased hepatic glycogen deposition
• increased peripheral insulin resistance
• increased gluconeogenesis
• increased free fatty acid production
• overall diabetogenic effect

Question 11

effects of excess cortisol on bone and calcium metabolism

Answer 11

• decreased bone formation

- decreased bone mass and osteoporosis

Question 12

effects of excess cortisol on immune system

Answer 12

• immunosuppression

- anti inflammatory action

Question 13

effects of excess cortisol on endocrine system

Answer 13

• decreased LH, FSH release
• decreased TSH
• decreased GH secretion

Question 14

effects of excess cortisol on skin/muscle tissue

Answer 14

• protein catabolism/collagen breakdown
• skin thinning
• muscular atrophy

Question 15

Primary Adrenal Insufficiency: Problem arises in the _____

Answer 15

adrenal cortex

Question 16

primary adrenal insufficiency decreases

Answer 16

cortisol and aldosterone

Question 17

autoimmune cause of primary adrenal insufficiency

Answer 17

Addisons disease

Question 18

infections that cause primary adrenal insufficiency

Answer 18

Tuberculosis, histoplasmosis, HIV

most common cause in some developing countries

Question 19

general causes of primary adrenal insufficiency

Answer 19

autoimmune, infectious, infiltrative, hemorrhage, drugs, inherited

Question 20

drugs that cause adrenal insufficiency

Answer 20

ketoconazole, rifampin

Question 21

enzyme defects impair cholesterol transport into adrenal glands

Answer 21

adrenal leukodystrophy

Question 22

histology of Addisons disease, early and late

Answer 22

early - lymphoid infiltration in cortex

late - fibrosis of cortex with spared medulla

Question 23

Spontaneous Bilateral Hemorrahage with Disseminated Infection

Answer 23

Waterhouse-Friderichsen Syndrome

Question 24

Features due to adrenal androgen deficiency

Answer 24

Decreased pubic and axillary hair (females)

Question 25

clinical features due to cortisol deficiency

Answer 25

``` Fatigue, anorexia, weight loss- 100% Hypotension- 88%; Postural symptoms- 12% Abdominal pain- 56% *Hyponatremia (dilutional)- 19% Hypoglycemia ```

Question 26

clinical features due to aldosterone deficiency

Answer 26

Hypovolemia, postural hypotension Salt craving *Hyperkalemia, *hyponatremia (salt loss) *Metabolic acidosis

Question 27

Features due to elevated ACTH (and concomittant elevated MSH)

Answer 27

Hyperpigmentation, esp skin folds, palm creases, buccal mucosa – 92%

Question 28

Secondary Adrenal Insufficiency: | Problem arises in ______

Answer 28

hypothalamus and/or pituitary

Question 29

#1 overall cause of secondary adrenal insufficiency!!

Answer 29

long term use exogenous glucocorticoids

Question 30

Sheehan’s syndrome

Answer 30

pituitary infarction

Question 31

If therapy is abruptly withdrawn, the atrophic adrenals cannot secrete adequate quantities of hormones which leads to

Answer 31

→ Seconday acute adrenal insufficiency

Question 32

In secondary AI, aldosterone secretion is____

Answer 32

preserved

Question 33

synthetic ACTH

Answer 33

Cosyntropin

Question 34

Secondary adrenal insufficiency treatment

Answer 34

glucocorticoid replacement - no need for mineralocorticoids

Question 35

Mineralocorticoid replacement examples

Answer 35

Hydrocortisone | Fludrocortisone

Question 36

why do we need mineralocorticoid replacement

Answer 36

As required to maintain normal BP and vascular volume | Serum potassium and sodium

Question 37

most potent glucocorticoid

Answer 37

Dexamethasone

Question 38

Patients using higher than physiologic doses of_________ (> equivalent of ~ 20 mg/day of HC) are at risk for ________ when steroids are withdrawn, in addition to the risks of chronic cortisol excess

Answer 38

glucocorticoids secondary AI

Question 39

Thus, when prescribing glucocorticoids for inflammatory or other disorders, use the ____ dose needed for the____ possible duration, and consider alternative treatments where possible.

Answer 39

lowest shortest

Question 40

Glucocorticoid replacement for primary adrenal insufficiency

Answer 40

- Hydrocortisone or prednisone - Equivalent to basal cortisol secretion of 20-30 mg/day - Increased dosing required during stress

Question 41

Group of autosomal recessive disorders characterized by mutation (and thus deficiency) of an enzyme in the adrenal steroid synthesis pathway.

Answer 41

congenital adrenal hyperplasia

Question 42

ACTH levels in congenital adrenal hyperplasia

Answer 42

high

Question 43

defective enzyme in 90 % of CAH

Answer 43

21-hydroxylase enzyme

Question 44

Clinical Manifestations of 21-hydroxylase Deficiency

Answer 44

cortisol and aldosterone deficiency adrenal androgen excess

Question 45

defective enzyme in 5% of CAH

Answer 45

11-hydroxylase Deficiency

Question 46

when 11-DOC is produced in large quantities due to 11-OHase deficiency, it exerts a marked mineralocorticoid effect, thus patients have ____

Answer 46

thus patients have hypertension

Question 47

cholesterol synthesis steps

Answer 47

cholesterol - > pregnenolone - > progesterone - > deoxycorticosterone - > corticosterone - > aldosterone

Question 48

adrenal insufficiency confirmed by

Answer 48

biochemical testing

Question 49

CAH- deficiencies in adrenal synthetic enzymes results in ____ cortisol and ______ adrenal gland size

Answer 49

decreased adrenal hyperplasia/increased adrenal gland size from increased ACTH