Peds Adrenal Flashcards
(26 cards)
What is the HPA axis?
A neuroendocrine system that controls reactions to stress and regulates digestion, the immune system, mood and emotions, sexuality, energy storage and expenditure
What is the short term stress response?
Involves stimulation of the adrenal medulla via preganglionic sympathetic fibers resulting in the release of catecholamines (E and NE)
What is the long term stress response?
Involves CRH stimulation of the anterior pituitary, stimulation of the adrenal cortex by ACTH and release of mineralocorticoids and glucocorticoids
What stimulates renin secretion from the kidney?
Decreased renal perfusion and/or increased sympathetic activity
What is the MCC of ambiguous genitalia in a genetically female infant?
CAH causing virilization of the genitalia
What are the signs and sx in a neonate with CAH?
Failure to thrive, recurrent vomiting, dehydration, hypotension, hyponatremia, hyperkalemia and shock
What are the mainstays of treatment in an infant in crisis due to CAH?
Hydrocortisone (IV or IM), fluids/glucose (IV) and management of hyperkalemia
What are the 3 categories of adrenal gland defects responsible for adrenal insufficiency (Addison dz)?
Adrenal dysfunction/destruction (autoimmune), adrenal dysgenesis, impaired steroidogenesis
What do nearly all pts with primary adrenal insufficiency complain of?
Fatigue, reduced stamina, weakness, anorexia, wt loss, skin hyperpigmentation
What do most pts with primary adrenal insufficiency complain of?
Abd pain, N/V, MSK pain, psych sx (depression, anxiety, irritability), HA, salt craving, low BP
What are the lab findings associated with primary adrenal insufficiency?
Moderate neutropenia, low serum Na, high serum K, fasting hypoglycemia, low 8am plasma cortisol accompanied by simultaneous significant elevation of plasma ACTH
What are the signs and sx of acute adrenal crisis?
Dehydration, dizziness (due to low BP), rapid HR and RR, confusion, N/V, fever, HA, abd pain
What are the lab findings associated with acute adrenal crisis?
Low serum cortisol, low blood sugar, low serum Na, high serum K, metabolic acidosis, inadequate bump in cortisol level with ACTH stimulation test
What is the treatment for acute adrenal crisis?
Hydrocortisone, fluids/glucose, fludrocortisone, and treat hyperkalemia if needed
Low serum ACTH in the setting of low serum cortisol is consistent with what?
Secondary adrenal insufficiency
An elevated midnight cortisol level is indicative of what?
Cushing syndrome
What is the best screening test for Cushing’s syndrome?
Dexamethasone stimulation test
What are the signs and sx of primary aldosteronism?
HTN, hypokalemia, muscle weakness, paresthesia with tetany, HA, polyuria/polydipsia
When should a dx of primary aldosteronism be considered?
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
What 3 things should be remembered for primary aldosteronism?
Low renin HTN, hypokalemia, metabolic alkalosis
What are paragangliomas?
Located outside the adrenal gland; secrete catecholamines (NE and E) or are non secreting
What are the sx of pheochromocytoma and paraganglioma (present similarly)?
Paroxysmal in timing, HTN, pounding HA, perspiration, panic (impending doom), palpitations, pallor
What is the most sensitive test for diagnosing secretory pheochromocytoma and paragangliomas?
Plasma fractionated free metanephrines
What is VHL type 2 dz?
AD disorder in which pts will develop pheochromocytoma, renal capillary hemangiomas/hemangioblastomas (+ CNS), and increased risk of renal cysts that transform into RCC
