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41 Cards in this Set
- Front
- Back
Layers of the adrenal cortex and what they secrete
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Zona glomerulosa - Mineralocorticoids (aldosterone), Zona Fasciculata - glucocorticoids (cortisol), Zona Reticularis - androgens (DHEA/S)
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What is the normal cycle of cortisol throughout the day?
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Rising starting around 3-4am, peaking at around 9am, falling until baseline at around 10-11pm.
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Which has more influence over aldosterone? RAAS or ACTH
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RAAS
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What would be the effect on Aldosterone, Cortisol, Androgens if you were ACTH Deficient? Had primary adrenal insufficiency? Panhypo pit?
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What is aldosterone's primary function?
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Regulate sodium and potassium balance. When acting, it increases sodium reabsorption and potassium secretion.
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What hormone will be high when there is excess adrenal sex steroids? What is the converted to?
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DHEA, DHEA-S
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What does excess adrenal sex steroids do for men and women?
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Not much in men, hirsutism, acne, clitoromegaly.
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Nausea, body aches, orthostatic hypotension are signs of what endocrine problem?
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Primary adrenal insufficiency
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Why would someone with adrenal insufficiency have hyperpigmentation?
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The POMC gene, which contains ACTH, also contains MSH. A large increase in ACTH then means a large increase in MSH.
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How will your Na+, K+, Glucose, and Cortisol react to primary adrenal insufficiency? Why?
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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).
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How do you test for low cortisol when you suspect primary adrenal insufficiency?
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Give synthetic ACTH - Cortrosyn IV and check cortisol levels after 1 hour.
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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?
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US: Autoimmune. World: TB. HIV patients can get it secondary to MAI or CMV. Ketoconazole can inhibit cortisol synthesis and simulate adrenal insufficiency.
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Tx of adrenal insufficiency - children, adults
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Children: Hydrocortisone (watch carefully for growth changes). Adults: Prednisone. Fludrocortisone for aldosterone replacement. Titrate to minimum effective dose.
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How to titrate doses of glucocorticoids
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Individualize - find the dose that keeps them from aching, make sure they're hitting milestones, then don't go much higher.
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Why do people with Addison's have salty sweat?
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Sweat glands have aldosterone receptors, which when stimulated reduce salt loss.
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Sick/Trauma management of adrenal insufficiency
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Sick: Double dose. Significant infection: Triple it. Major trauma: Quadruple it.
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What is hyporeninemic hypoaldosteronism?
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Problem with JG apparatus, causing decreased function of renin typically in DM2 patients. Precursor to renal insufficiency.
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How can you treat hypotensive patients that are unresponsive to fluids?
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Steroids. Also, epi/norepi won't work until you have steroids on board, because they are a requisite factor.
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What is the relative strength of these steroids: Hydrocortisone, Prednisone, Dexamethasone
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From least to most potent: Hydro, Pred, Dexa
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How can you check for Secondary Adrenal insufficiency?
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Combine an insulin bolus (decrease blood sugar, causes pituitary to release ACTH) with history. You don't need to do two tests.
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Physical findings for Cushing's syndrome
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Plethora, Supraclavicular fat pad, Thoracodorsal fat pad, Purple striae
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Four possible etiologies for Cushing Syndrome
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Pituitary adenoma, Ectopic ACTH, Adrenal adenoma, Exogenous steroids
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Three possible tests for Cushing syndrome
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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).
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What to do with a positive result on the cortisol test?
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Confirm: Urine free cortisol OR slug of dexamethasone (2 mg) to be sure to shut off the pituitary. (But an endocrinologist will do this)
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With a positive result for excess cortisol, what is the most common outcome?
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Cushing Disease (Pituitary adenoma)
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How might you differentiate between Cushing's disease and an ectopic ACTH tumor?
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Ectopic will typically have low K+ as well.
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Tx of Cushing's Disease
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Transphenoidal resection (followed by irradiation and chemo)
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Treatment of Adrenal Tumors (Cushing Syndrome w/o ACTHoma)
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Unilateral adrenalectomy. If you can't operate, take Mitotane (adrenal toxin, reduces all steroids), also Ketoconazole.
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How does an adrenal carcinoma act?
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Junk tumor - secretes everything, but cortisol is the biggest problem.
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MOA of Mitotane
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Permanent destruction of adrenal cells.
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MOA of Ketoconazole in the context of adrenal tumor
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Cortisol-reducing effects in higher doses.
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What is the 21 Hydroxylase defect? What does it cause? Common sxs.
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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.
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In what population is 21 Hydroxylase defect most common in?
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Ashkenazi jews.
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How is 21 Hydroxylase defect treated?
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Small amounts of cortisol inhibit ACTH and rectify the lack of cortisol. Mostly fixes everything.
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Classical vs. Nonclassical 21 Hydroxylase defect
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Classical - full deficiency, shows up in small children, screened for. Nonclassical - partial deficiency, shows up later, not screened for.
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How can you check for 21 Hydroxylase defect?
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Check baseline 17-OH Progesterone (during follicular phase) against ACTH stimulated by Cortrosyn. Higher levels indicate more blockade.
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What is a pheochromocytoma? Sxs? Screen/Dx? Tx?
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A catecholamine secreting tumor. Sxs: Headaches, Palpitations, Sweating, Nausea, Anxiety, FID. Elevated urine metanephrines. Alpha then Beta blockade followed by complete adrenalectomy.
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Following adrenalectomy for a pheochromocytoma, what will happen?
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BP will drop and volume expands d/t sudden lack of catecholamines.
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What is the biochemical workup for an incidental adrenal mass?
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Urine metanephrines - for pheochromocytoma. Overnight dexamethasone - for cortisol secreting tumor. Renin/Aldosterone ratio - for aldosterone secreting adenoma. No screen for sex steroid.
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Incidentaloma size and what to do
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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.
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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?
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It might be one of the crazy familial forms of aldosteronism.
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