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41 Cards in this Set

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Layers of the adrenal cortex and what they secrete
Zona glomerulosa - Mineralocorticoids (aldosterone), Zona Fasciculata - glucocorticoids (cortisol), Zona Reticularis - androgens (DHEA/S)
What is the normal cycle of cortisol throughout the day?
Rising starting around 3-4am, peaking at around 9am, falling until baseline at around 10-11pm.
Which has more influence over aldosterone? RAAS or ACTH
RAAS
What would be the effect on Aldosterone, Cortisol, Androgens if you were ACTH Deficient? Had primary adrenal insufficiency? Panhypo pit?

What is aldosterone's primary function?
Regulate sodium and potassium balance. When acting, it increases sodium reabsorption and potassium secretion.
What hormone will be high when there is excess adrenal sex steroids? What is the converted to?
DHEA, DHEA-S
What does excess adrenal sex steroids do for men and women?
Not much in men, hirsutism, acne, clitoromegaly.
Nausea, body aches, orthostatic hypotension are signs of what endocrine problem?
Primary adrenal insufficiency
Why would someone with adrenal insufficiency have hyperpigmentation?
The POMC gene, which contains ACTH, also contains MSH. A large increase in ACTH then means a large increase in MSH.
How will your Na+, K+, Glucose, and Cortisol react to primary adrenal insufficiency? Why?
Na+ (low d/t lack of aldosterone production), K+ (high d/t lack of aldosterone), Glucose (low d/t gluconeogenesis from cortisol), Cortisol (low in the morning).
How do you test for low cortisol when you suspect primary adrenal insufficiency?
Give synthetic ACTH - Cortrosyn IV and check cortisol levels after 1 hour.
What is the leading U.S. cause of primary adrenal insufficiency? In the world? What may cause primary adrenal insufficiency in HIV patients? What drug could induce it?
US: Autoimmune. World: TB. HIV patients can get it secondary to MAI or CMV. Ketoconazole can inhibit cortisol synthesis and simulate adrenal insufficiency.
Tx of adrenal insufficiency - children, adults
Children: Hydrocortisone (watch carefully for growth changes). Adults: Prednisone. Fludrocortisone for aldosterone replacement. Titrate to minimum effective dose.
How to titrate doses of glucocorticoids
Individualize - find the dose that keeps them from aching, make sure they're hitting milestones, then don't go much higher.
Why do people with Addison's have salty sweat?
Sweat glands have aldosterone receptors, which when stimulated reduce salt loss.
Sick/Trauma management of adrenal insufficiency
Sick: Double dose. Significant infection: Triple it. Major trauma: Quadruple it.
What is hyporeninemic hypoaldosteronism?
Problem with JG apparatus, causing decreased function of renin typically in DM2 patients. Precursor to renal insufficiency.
How can you treat hypotensive patients that are unresponsive to fluids?
Steroids. Also, epi/norepi won't work until you have steroids on board, because they are a requisite factor.
What is the relative strength of these steroids: Hydrocortisone, Prednisone, Dexamethasone
From least to most potent: Hydro, Pred, Dexa
How can you check for Secondary Adrenal insufficiency?
Combine an insulin bolus (decrease blood sugar, causes pituitary to release ACTH) with history. You don't need to do two tests.
Physical findings for Cushing's syndrome
Plethora, Supraclavicular fat pad, Thoracodorsal fat pad, Purple striae
Four possible etiologies for Cushing Syndrome
Pituitary adenoma, Ectopic ACTH, Adrenal adenoma, Exogenous steroids
Three possible tests for Cushing syndrome
Gold standard: Suppress ACTH with dexamethasone (1 mg), if the AM cortisol is high, test is +. 24 hour urine cortisol (test for cortisol in urine). Late night salivary test: Test salivary cortisol (x2 nights).
What to do with a positive result on the cortisol test?
Confirm: Urine free cortisol OR slug of dexamethasone (2 mg) to be sure to shut off the pituitary. (But an endocrinologist will do this)
With a positive result for excess cortisol, what is the most common outcome?
Cushing Disease (Pituitary adenoma)
How might you differentiate between Cushing's disease and an ectopic ACTH tumor?
Ectopic will typically have low K+ as well.
Tx of Cushing's Disease
Transphenoidal resection (followed by irradiation and chemo)
Treatment of Adrenal Tumors (Cushing Syndrome w/o ACTHoma)
Unilateral adrenalectomy. If you can't operate, take Mitotane (adrenal toxin, reduces all steroids), also Ketoconazole.
How does an adrenal carcinoma act?
Junk tumor - secretes everything, but cortisol is the biggest problem.
MOA of Mitotane
Permanent destruction of adrenal cells.
MOA of Ketoconazole in the context of adrenal tumor
Cortisol-reducing effects in higher doses.
What is the 21 Hydroxylase defect? What does it cause? Common sxs.
It is an enzyme that converts precursors to cortisol (and aldosterone). Without it, cortisol production gets shunted to testosterone production, and as a result there is nothing inhibiting ACTH. Sxs: Women - hirsutism, infertility, acne, male pattern baldness. Men - adrenal rest tissue in gonads, oligospermia. Common in Ashkenazi jews.
In what population is 21 Hydroxylase defect most common in?
Ashkenazi jews.
How is 21 Hydroxylase defect treated?
Small amounts of cortisol inhibit ACTH and rectify the lack of cortisol. Mostly fixes everything.
Classical vs. Nonclassical 21 Hydroxylase defect
Classical - full deficiency, shows up in small children, screened for. Nonclassical - partial deficiency, shows up later, not screened for.
How can you check for 21 Hydroxylase defect?
Check baseline 17-OH Progesterone (during follicular phase) against ACTH stimulated by Cortrosyn. Higher levels indicate more blockade.
What is a pheochromocytoma? Sxs? Screen/Dx? Tx?
A catecholamine secreting tumor. Sxs: Headaches, Palpitations, Sweating, Nausea, Anxiety, FID. Elevated urine metanephrines. Alpha then Beta blockade followed by complete adrenalectomy.
Following adrenalectomy for a pheochromocytoma, what will happen?
BP will drop and volume expands d/t sudden lack of catecholamines.
What is the biochemical workup for an incidental adrenal mass?
Urine metanephrines - for pheochromocytoma. Overnight dexamethasone - for cortisol secreting tumor. Renin/Aldosterone ratio - for aldosterone secreting adenoma. No screen for sex steroid.
Incidentaloma size and what to do
Less than 4 cm, CT again in 6 months (and then annually) and compare size. Greater than 6 cm, surgery unless it looks benign AND has no associated symptoms.
If you have high aldosterone levels which you can't suppress with dexamethasone, but you also can't anything on CT, what might it be?
It might be one of the crazy familial forms of aldosteronism.