A.I. – Aye Yai Yai

by Elizabeth Perry, MD

 

35 yo F presents to your Resus Room. She’s hypotensive, and not really responding to fluids, and have tried almost all of your pressors. You find out from her accompanying family members that she’s an asthmatic, steroid dependent, and hasn’t been able to make her insurance payments on time as of recent.

What’s going on? (hint: adrenal insufficiency)

A brief reminder of the adrenal gland:
Synthesizes steroid hormones (glucocorticoids – cortisol, mineralocorticoid – aldosterone, gonadocorticoids – testosterone, estrogen) in the cortex and catecholamines (epinephrine, norepinephrine, dopamine) in the cortex.

Primary Adrenal Gland Insufficiency = Addison’s
-destruction of > 90% of adrenal glands
-Results in decreased cortisol and aldosterone production
-rare
-causes: autoimmune destruction, hemorrhage (from use of warfarin,sepsis, trauma), tumor (breast and melanoma), infection (HIV, Tb, meningococcemia) or inflammatory process.
-In US 70% of primary AI is due to autoimmune disorders. Can be isolated or associated with polyglanduar autoimmune syndrome (PGA) type I or II.
About 20% of HIV patients eventually develop adrenal insufficiency, most common infectious cause in US. Worldwide most common infectious cause is TB.

Secondary Adrenal Gland Insufficiency
-insufficient production of ACTH. Occurs following disorders in the hypothalamic–pituitary axis, when CRF and/or ACTH fails to be secreted.
most common cause is suppression of HPA axis over time due to long term therapy with pharmacologic doses of glucocorticoids.
-other causes: destruction or dysfunction of the pituitary (pituitary disease, head trauma, postpartum pituitary necrosis- Sheehan Syndrome)
-mineralocorticoid (aldosterone) function intact, loss of glucocorticoid (cortisol) activity

Tertiary Adrenal Gland Insufficiency
-hypothalamic disease and suppression of CRH

ADRENAL CRISIS
The life–threatening exacerbation of adrenal insufficiency due to increased physiologic demand (infection) or decreased supply (discontinuation of steroid therapy) of cortisol. Usually occurs in response to major stress: MI, sepsis, surgery, major injury, trauma.

Symptoms:
GI: abdominal pain, vomiting and diarrhea
CVS: dehydration, hypotension, refractory shock, poor response to inotropes/pressors
fever
confusion

TREATMENT OF ADRENAL CRISIS
fluid resuscitation (recommendation is D5NS for both hyponatremia and hypoglycemia)
-reversal of electrolyte abnormalities
high dose hydrocortisone (100mg IV Q6 hrly): provides both glucocorticoid and mineralocorticoid effects
-IV Dexamethasone 4mg may be given if the rapid ACTH stimulation test will be part of diagnostic workup
vasopressors in patients unresponsive to fluid resuscitation (norepinephrine, dopamine, phenylephrine preferred)

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