Peds Adrenal

Question 1

What is the HPA axis?

Answer 1

A neuroendocrine system that controls reactions to stress and regulates digestion, the immune system, mood and emotions, sexuality, energy storage and expenditure

Question 2

What is the short term stress response?

Answer 2

Involves stimulation of the adrenal medulla via preganglionic sympathetic fibers resulting in the release of catecholamines (E and NE)

Question 3

What is the long term stress response?

Answer 3

Involves CRH stimulation of the anterior pituitary, stimulation of the adrenal cortex by ACTH and release of mineralocorticoids and glucocorticoids

Question 4

What stimulates renin secretion from the kidney?

Answer 4

Decreased renal perfusion and/or increased sympathetic activity

Question 5

What is the MCC of ambiguous genitalia in a genetically female infant?

Answer 5

CAH causing virilization of the genitalia

Question 6

What are the signs and sx in a neonate with CAH?

Answer 6

Failure to thrive, recurrent vomiting, dehydration, hypotension, hyponatremia, hyperkalemia and shock

Question 7

What are the mainstays of treatment in an infant in crisis due to CAH?

Answer 7

Hydrocortisone (IV or IM), fluids/glucose (IV) and management of hyperkalemia

Question 8

What are the 3 categories of adrenal gland defects responsible for adrenal insufficiency (Addison dz)?

Answer 8

Adrenal dysfunction/destruction (autoimmune), adrenal dysgenesis, impaired steroidogenesis

Question 9

What do nearly all pts with primary adrenal insufficiency complain of?

Answer 9

Fatigue, reduced stamina, weakness, anorexia, wt loss, skin hyperpigmentation

Question 10

What do most pts with primary adrenal insufficiency complain of?

Answer 10

Abd pain, N/V, MSK pain, psych sx (depression, anxiety, irritability), HA, salt craving, low BP

Question 11

What are the lab findings associated with primary adrenal insufficiency?

Answer 11

Moderate neutropenia, low serum Na, high serum K, fasting hypoglycemia, low 8am plasma cortisol accompanied by simultaneous significant elevation of plasma ACTH

Question 12

What are the signs and sx of acute adrenal crisis?

Answer 12

Dehydration, dizziness (due to low BP), rapid HR and RR, confusion, N/V, fever, HA, abd pain

Question 13

What are the lab findings associated with acute adrenal crisis?

Answer 13

Low serum cortisol, low blood sugar, low serum Na, high serum K, metabolic acidosis, inadequate bump in cortisol level with ACTH stimulation test

Question 14

What is the treatment for acute adrenal crisis?

Answer 14

Hydrocortisone, fluids/glucose, fludrocortisone, and treat hyperkalemia if needed

Question 15

Low serum ACTH in the setting of low serum cortisol is consistent with what?

Answer 15

Secondary adrenal insufficiency

Question 16

An elevated midnight cortisol level is indicative of what?

Answer 16

Cushing syndrome

Question 17

What is the best screening test for Cushing’s syndrome?

Answer 17

Dexamethasone stimulation test

Question 18

What are the signs and sx of primary aldosteronism?

Answer 18

HTN, hypokalemia, muscle weakness, paresthesia with tetany, HA, polyuria/polydipsia

Question 19

When should a dx of primary aldosteronism be considered?

Answer 19

When a pt has tx resistant HTN, severe HTN, early onset HTN, HTN with an adrenal mass, low renting HTN, HTN with a FHx of early onset HTN or CVA, 1st degree relative with aldosteronism

Question 20

What 3 things should be remembered for primary aldosteronism?

Answer 20

Low renin HTN, hypokalemia, metabolic alkalosis

Question 21

What are paragangliomas?

Answer 21

Located outside the adrenal gland; secrete catecholamines (NE and E) or are non secreting

Question 22

What are the sx of pheochromocytoma and paraganglioma (present similarly)?

Answer 22

Paroxysmal in timing, HTN, pounding HA, perspiration, panic (impending doom), palpitations, pallor

Question 23

What is the most sensitive test for diagnosing secretory pheochromocytoma and paragangliomas?

Answer 23

Plasma fractionated free metanephrines

Question 24

What is VHL type 2 dz?

Answer 24

AD disorder in which pts will develop pheochromocytoma, renal capillary hemangiomas/hemangioblastomas (+ CNS), and increased risk of renal cysts that transform into RCC

Question 25

What is the tx for pheochromocytoma?

Answer 25

Treat with alpha blocker first and then beta beta blockers can be started; never start beta blocker first as unopposed alpha receptor stimulation can lead to further elevated BP

Question 26

What do pts with adrenal incidentalomas require?

Answer 26

Clinical assessment for Cushing syndrome, hyperaldosteronism and testing for pheochromocytoma