Lecture 22 - Clinical Aspects of Adrenal Disorders

Question 1

The adrenal medulla is derived from ____ ____ cells, while the cortex is _______ derived.

Answer 1

Neural Crest

Mesoderm

Question 2

_______ blocks 11-hydroxylase activity, thus it is a drug that can lead to Primary Adrenal Insufficiency.

Answer 2

Ketoconazole

Question 3

Polyglandular Autoimmune Syndrome Type 1 we remember results from a mutation in the _____ gene –> this distorts FOXP3 function, which is important for producing regulatory T-cells.

Answer 3

AIRE

Question 4

Plasma Cortisol (PC) levels < _____micrograms/dL is diagnostic of Addison’s.

PC > ____ micrograms/dL is normal.

For acutely ill patients, PC Less than or equal to ____micrgrams/dL is diagnostic of Addison’s and PC less than or equal to _____ is presumptive.

For acutely ill patients, PC values between 13-18 require interim _______ therapy while awaiting results of ______ stimulation test.

Answer 4

3

18

5

13

Glucocorticoids

Question 5

Plasma ACTH in Primary Adrenal Insufficiency is invariably > _____pg/mL.

Answer 5

100

Question 6

_______ (synthetic ACTH) is used for the ACTH stimulation test to detect Addison’s. Serum Cortisol < _____micrograms/dL at the 30min or 60min mark OR an increase of < _____micrograms/dL from baseline are indicative of Addison’s.

Answer 6

Cosyntropin

18

9

Question 7

For Addison’s patients who are Negative for Abs and who are Male, check plasma _______ –> if test is positive, this is diagnostic of _________.

Answer 7

VLCFA (Very long chain fatty acids)

Adrenoleukodystrophy

Question 8

Screening for Cushing’s often involves 24hr urine collection. Cortisol above ______micrograms/dL is abnormal.

Also, overnight dexamethasone (1mg) test can be used –> morning fasting Cortisol is measured –> if > _____micrograms/dL, additional testing needed.

Late-night salivary cortisol (LNSC) can also be used to measure free cortisol –> if it’s elevated, then there’s loss of ____ rhythm.

Answer 8

50

1.8

Diurnal rhythm

Question 9

Low serum ACTH is considered < ____ pg/mL.

Answer 9

5pg/mL

Question 10

High dose Dexamethasone suppression test is conducted with ____mg dexamethasone. If morning fasted cortisol level is < ____% of baseline, then Pituitary is the cause.

Answer 10

8mg

50%

Question 11

______ is given to Cushing’s patients as prophylactic protection against opportunistic pathogens.

Answer 11

Bactrim

Question 12

Carney’s Triad (GI stromal tumor, pulmonary chondroma, and extra-adrenal paraganglioma) is associated with Pheochromocytoma, and it arises from a mutation in ______.

Answer 12

SDHB (Succinate dehydrogenase B)

Question 13

Remember rule of ____% in pheochromocytomas.

Answer 13

10%

Question 14

_____ 68 dotate is the best scan for localizing pheochromocytoma.

Answer 14

Gallium 68 dotate

Question 15

Plasma free ______ is the screening of choice for Pheochromocytoma.

Answer 15

Metanephrines

Question 16

The following are all reasons to investigate Hyperaldosteronism in a patient with HTN.

Spontaneous Hypokalemia with serum K < ____mEq/L.

Hypokalemia < ____mEq/L despite K supps or K sparing diuretics.

Failure to normalize K within ___ weeks of diuretic cessation.

Answer 16

3.5

3

4

Question 17

Aldosterone : Renin ratio (ARR) > ______ is considered elevated –> indicative of Primary Hyperaldosteronism. However, a high ARR can result from HYPER______ –> stimulates Aldosterone without Renin.

Answer 17

25

Hyperkalemia