Adrenal Gland Red Highlights - Newman

Question 1

Main signs in primary Adrenal Insufficiency?

Answer 1

Skin hyperpigmentation
Salt craving
Increased ACTH

Question 2

Imaging for ACTH-independent Cushing Syndrome?

Dependent?

Answer 2

CT of adrenals

MRI of pituitary

Question 3

Low serum ACTH in the setting of low serum cortisol indicates what?

What sx?

Answer 3

Secondary adrenal insufficiency

Alabaster skin

Question 4

Addison’s disease results in decreased what?

Despite increased what?

Answer 4

DEC mineralcorticoids, glucocorticoids, and androgens

ACTH

Question 5

ACTH-independent Cushing Syndrome causes?

Answer 5

Exogenous corticosteroid use

Adrenocortical tumor

Question 6

What are the 3 causes of adrenal insufficiency in order of most to least common?

Answer 6

1. Adrenal destruction
2. Adrenal dysgenesis
3. Impaired steroidogenesis

Question 7

NE causes what sx?

Epi?

Answer 7

HTN

tachyarrythmias

Question 8

What should you test for in pts w/HTN and hypokalemia, severe HTN, early onset HTN, or tx-resistant HTN?

Answer 8

Primary aldosteronism

Question 9

Pheos secrete what?

Paragangliomas?

Answer 9

Epi and NE

Only NE

Question 10

What is the tx for adrenal insufficiency?

Answer 10

Glucocorticoid replacement therapy: hydrocortisone, prednisone, prednisolone
Mineralcorticoid replacement therapy: fludrocortisone, acute (Na sparing)
DHEA: give to some women

Question 11

Pheochromacytomas and paragangliomas are tumors of what?

Answer 11

Sympathetic nervous system

Question 12

Imaging of pheo if suspected?

Answer 12

noncontrast CT of abdomen

Question 13

Inheritance of VHL type 2?

Major clinical features?

Answer 13

AD

Hemangioma of retina, cerebellum, brainstem
Pheos

Question 14

How do you treat Acute Adrenal crisis?

Answer 14

Hydrocortisone

Fluids

Question 15

What are discovered incidentally on abdominal CT or MRI?

When should they be resected?

Answer 15

Adrenal nodules –> adrenal incidentaloma

> 4cm

Question 16

During surgery of pheos and paragangliomas the abdomen can be seeded and result in multifocal recurrent intra-abdominal tumors, what is this called?

Answer 16

Pheochromocytomatosis

Question 17

MEN syndromes inheritance?

Answer 17

AD

Question 18

What is the confirmatory test in primary adrenal insufficiency?

Answer 18

Cosyntropin (synthetic ACTH) stimulation test

If cortisol levels do not respond to cosyntropin admin, it confirms AI

Question 19

Diagnosis?

Cortisol high
ACTH low

Answer 19

Tumor in adrenal gland

Question 20

Diagnosis?

Cortisol low
ACTH high

Answer 20

Damage to the adrenal gland

Question 21

What is impaired in Cushing syndrome?

What may indicate Cushing syndrome?

Easiest screening test?

Answer 21

Glucose tolerance

Elevated midnight cortisol levels

Dexamethasone suppression test

Question 22

If the ACTH is elevated in the hundred and the cortisol is NOT suppressed by high or low doses by dexamethasone, what does it mean?

Answer 22

Ectopic ACTH syndrome

Question 23

If the ACTH is undetectable or low and cortisol is NOT suppressed by high or low doses, what is the interpretation?

Answer 23

Primary hypercortisolism is likely

Question 24

Medical tx prior to resection of pheo?

Answer 24

Alpha-blocker –> Phenoxybenzamine

Question 25

Main ACTH-dependent Cushing syndrome?

Answer 25

Cushing disease | Small cell carcinoma of the lung

Question 26

All patients (even normotensive) w/an adrenal incidentaloma require testing for what?

Answer 26

Pheochromocytoma w/metanephrines

Question 27

What accounts for adrenal destruction in Addison's disease?

Answer 27

#1 autoimmune X-linked recessive disorder Infection --> TB B/l adrenal hemorrhage

Question 28

Diagnosis? Cortisol high ACTH high

Answer 28

Tumor in anterior pituitary

Question 29

All pts w/biochemically confirmed primary aldosteronism require what?

Answer 29

Thin-section CT scan of the adrenals to screen for adrenal carcinoma

Question 30

Can surgical histopathology for pheos determine malignancy?

Answer 30

NO

Question 31

In 21-hydroxylase deficiency, what is required for diagnosis?

Answer 31

Elevated serum levels of 17-OH progesterone

Question 32

Diagnosis? Cortisol low ACTH low

Answer 32

Hypopituitary adrenal insufficiency

Question 33

What is the single most sensitive test for secretory pheos and paragangliomas? What is elevated in serum 90% of the time?

Answer 33

Plasma fractionated free metanephrines Serum chromogranin A (CgA)

Question 34

Female infants w/21-hydroxylase deficiency will have what?

Answer 34

Virilized genitalia

Question 35

What accounts for adrenal dysgenesis?

Answer 35

Familial glucocorticoid deficiency (AR) Less important: Triple A (Allgrove syndrome) and congenital adrenal hypo-plasma

Question 36

What causes inappropriately high aldosterone secretion that does not suppress adequately w/sodium loading?

Answer 36

Primary aldosteronism

Question 37

Conn syndrome pts have primary aldosteronism secondary to what? 40% have a mutation in what? What complications frequently occur?

Answer 37

Unilateral aldosterone-producing adrenal adenoma Gene coding for K+ channel CVA

Question 38

What accounts for impaired steroidogenesis?

Answer 38

CAH | Smith-Lemli-Opitz (cholesterol biosynthesis disorder)