[CLMD] Pediatric endocrine; Adrenal Case

Question 1

Describe the HPA axis for cortisol

Answer 1

Question 2

What are the layers of the adrenal cortex?

What do they produce?

Answer 2

” Go Find Rex; Make Good Sex”

Glomerulosa, Fasciculata, Reticularis

Mineralocorticoid, glucocorticoid, sex steriods

Question 3

What is the cause of cushing syndrome?

Answer 3

Ecessive corticosteroids

Question 4

What category does McCune-Albright syndrome fall under?

Answer 4

ACTH independent cushing syndrome

Question 5

What lab tests do you administer for cushing syndrome?

Answer 5

Dexamethasone suppression test

Question 6

When performing a dexamethoasone suppression test, when do you take a serum ACTH?

Answer 6

Take a serum ACTH BEFORE dexamethasone is given

Question 7

Describe what the interpretation of the following lab results should be:

ACTH [undetectable/low], Cortisol [Not suppressed]

ACTH [elevated in the hundreds], Cortisol [Not suppressed]

ACTH [normal to elevated], Cortisol [Not suppressed]

Answer 7

ACTH [undetectable/low], Cortisol [Not suppressed] = Primary hypercortisolism

ACTH [elevated in the hundreds], Cortisol [Not suppressed] = Ectopic ACTH

ACTH [normal to elevated], Cortisol [Not suppressed] = Cushing “disease”

Question 8

Question 9

Describe how a healthy individual would respond to a ACTH (cosyntropin) stimulation test:

Answer 9

Administer 250 mcg cosyntropin

Cortisol level should increase above 18-20 microgram/dl within 60 min

Question 10

Describe how a primary adrenal insufficiency (addison’s disease) would respond to a ACTH (cosyntropin) stimulation test:

Answer 10

Administer 250 mcg cosyntropin

Cortisol will NOT rise during the test

Question 11

What is the cause of addision’s disease (autoimmune primary adrenal insufficiency)?

Answer 11

Autoimmune destruction of the adrenal cortex

Question 12

What are the hallmark symptoms of addison’s disease?

Answer 12

Lightheadedness

Weight loss

Orthostatic hypotension

Skin hyperpigmentation

Irregular menses or amenorrhea

Question 13

Why do addison’s patients get hyperpigmentation?

Answer 13

Increased ACTH leads to increased melanocyte stimulating hormone (MSH) which leads to hyperpigmentation

Question 14

What is the treatment for Addison’s disease?

Answer 14

Replace cortisol and aldosterone

Question 15

What is the most common enzyme deficiency in congenital adrenal hyperplasia?

Answer 15

21 Hydroxylase Deficiency

Question 16

In addition to autoimmune causes, what else can induce adrenal destruction?

Answer 16

TB

Waterhouse-Friderichsen syndrome

Question 17

What is the trick to remembering congenital adrenal hyperplasia values? (increased or decreased)

Answer 17

Anything with a “1” gets an up arrow

Everything else gets a down arrow

Question 18

What is primary aldosteronism?

Answer 18

Inappropriatley high aldosterone secretion that does not suppress adequatley with sodium loading

Question 19

What is Conn syndrome?

Answer 19

Primary aldosteronism secondary to a unilateral aldosterone-producing adrenal adenoma

Question 20

Pheochromocytomas and non-head-neck paragangliomas are tumors of the _____________

Answer 20

Pheochromocytomas and non-head-neck paragangliomas are tumors of the sympathetic nervous system

Question 21

What are the symptoms of a pheochromocytoma?

Answer 21

Paroxysymal

Pressure increase (blood)

Pounding pain (HA)

Perspiration

Panic

Palpitations

Pallor

Question 22

What test do you order with a suspicion of pheochromocytoma?

Answer 22

Plasma fractionated free metanephrines