Adrenal Disorders

glucocorticoids (cortisol), cortex

Cushing’ s syndrome/disease is a overproduction of __________ in the ____________

inhibit glucose uptake/metabolism, decrease protein synthesis, increase release of AA, lactate

What effect does cortisol have on the muscles

increase lipolysis

What is the effect of cortisol on the fat?

suppression, anti-inflammatory

What is the effect of cortisol on the immune system?

increase CO, increase peripheral vascular tone (HTN)

What is the effect of cortisol on the cardiovascular system?

increase gluconeogenesis, increase glycogen synthesis

What is the effect of cortisol on the liver

increase GFR, aid in water regulation and electrolyte balance

What is the effect of cortisol on the renal system?

increase blood glucose

Other effects of cortisol

Cushing syndrome

clinical manifestations (S/S) due to excessive glucocorticoids either from exogenous steroid use or spontaneous production of excess corticosteroids by the adrenal cortex

Cushing Disease

ACTH hypersecretion from the anterior pituitary due to a benign pituitary adenoma

central obesity, buffalo hump, supraclavicular fat pad, moon fascies

Cushings presentation - weight gain

purple striae (loss of collagen), easy bruising, thin skin, slow wound healing, unusual bacterial/funga; infections

Cushings presentation - Skin changes

proximal muscle weakness, emotional lability, depression, osteoporosis, HA (pituitary tumor)

Cushings presentation - Neuro and musculoskeletal

HTN

Cushings presentation - CV and renal

DM, menstrual irregularities, decrease libido, infertility, ED

Cushings presentation - Endrocrine

Serum and urinary cortisol, ACTH, CMP (glucose, K), UA (glucose), CBC (leukocytosis)

35 y/o female presents to the clinic for muscle weakness and fatigue. On a physical exam you note central obesity, purple striae on the upper extremities and abdomen. Vitals are stable with the exception of 150/90. What labs do you want?

Dexamethasone suppression test, 24 hour urine, midnight serum/salivary cortisol levels

35 y/o female presents to the clinic for muscle weakness and fatigue. On a physical exam you note central obesity, purple striae on the upper extremities and abdomen. Vitals are stable with the exception of 150/90. Labs show elevated serum and urinary cortisol, glycosuria, hypokalemia, and leukocytosis. What are some tests you can use to confirm your diagnosis of Cushings?

Give Dex at 11 pm, check cortisol in the morning if lower than 1.8 the pituitary responded and cushings is excluded

Describe the dexamethasone suppression test

Adrenal CT (adrenal tumor)

In a Cushing’s workup, if serum ACTH is low, what imaging do you want

Pituitary MRI (pituitary tumor), CT of chest/abdomen (ectopic tumor)

In a Cushing’s workup, if serum ACTH is high, what imaging do you want

transphenoidal selective resection of pituitary adenoma

Treatment of choice for Cushing’s disease (ACTH dependent)

unilateral adrenalectomy

Treatment plan for adrenal adenoma/ACTH independent hyercortisolism

hydrocortisone replacement (due to delay in normal CRH/ACTH secretion - then taper it down)

How can we avoid adrenal crisis after treating Cushing’s?

ketoconazole (adrenal enzyme inhibitors)

If surgery is not an option for endogenous Cushing syndrome, what is our 1st line medication?

Adrenocortical insufficiency (Addison’s disease)

Progressive hypofunctioning of the adrenal cortex

mineralcorticoids, glucocorticoids (cortex)

In addison’s disease, what are we deficient in

autoimmune destruction (90% of cases in US), infectious (TB, HIV, CMV), bilateral adrenal hemorrhage, congenital adrenal hyperplasia

What are some causes of Primary adrenal insufficiency

fatigue, anorexia, weight loss, N/V/D, abdominal pain, arthralgias, myalgias, hypotension

What are the most common symptoms of primary adrenal insufficiency?

tachycardia, decreased axillary hair and libido in women, irritability, depression, hyperpigmentation (usually shows up 1st), salt cravings

Less common symptoms of primary adrenal insufficiency

Low AM cortisol, ACTH over 200, low Na, high K, low serum DHEA, anti-adrenal antibodies, elevated plasma renin activity

Labs for primary adrenal insufficiency

Chest CT (TB, fungal infection, cancer), CT adrenals (1st draft pick), FNA (determines etiology)

Imaging for primary adrenal insufficiency

Hydrocortisone (glucocorticoid replacement - could use dex, prednisone), Fludrocortisone (mineralcorticoid replacement), DHEA (in women with refractory symptoms)

Treatment for primary adrenal insufficiency

Stress dose of glucocorticoids for 3 days

What should be done for a patient with adrenal insufficiency who encounters a stressful situation (fever, surgery, etc)

Stress dose IV hydrocortisone 100-300 mg, NS rapid bolus (D50 if hypoglycemic), empiric antibiotics

45 y/o woman presents to the ER with complaints of N/V and abd pain. Vitals are stable with the exception of 90/50 and 104.3 temp. Labs show hyponatremia, hyperkalemia, and hypoglycemia. What is your treatment plan?

hydrocortisone

What med do you prioritize in an adrenal crisis

Pheochromocytoma

A tumor in the adrenal medulla that secretes both norepi and epi

HA, palpitations/tachy, diaphoresis

Pheo classic triad

sustained/paroxysmal HTN, orthostatic hypotension, tremor

Other symptoms of pheo

High plasma free metanephrines (most sensitive), urine VMAs, abdominal CT/MRI, nuclear imaging

Diagnostics for pheo

adrenalectomy (1st draft pick), get pressure under control before surgery with alpha blockers or CCBs, Tachycardia can be controlled with a beta blocker AFTER HTN is undercontrol

25 y/o male patient presents to the ER for palpitations stating it feels like “his heart is racing out of his chest.” He also reports HA. On a physical exam you note HTN (180/126), 176 bpm, skin is diaphoretic, and a slight tremor of the hands is noted. Labs show high plasma free metanephrines and high urine VMAs. What is your treatment plan?

Primary aldosteronism

What is caused by the autonomous production of aldosterone (mineralocorticoid) by the adrenal cortex due to hyperplasia, adenoma, or carcinoma

increases water retention, increasing Na+ retention

What is the function of aldosterone

Bilateral idiopathic hyperaldosteronism, unilateral aldosterone producing adenoma, unilateral hyperplasia, pure aldosterone producing adrenocortical carcinomas and ectopic aldosterone-secreting tumors

Causes of primary hyperalsoteronism

dehydration, CHF, Cirrhosis, Nephrotic syndrome

Causes of Secondary hyperaldosteronism

Resistant HTN, Hypokalemia (muscle weakness), mild hypernatremia

Symptoms of primary hyperaldosteronism

HTN with hypokalemia, Resistant HTN (3+ meds to manage), Severe HTN, onset of HTN under 30 y/o, HTN with adrenal mass, Family Hx of early onset cardiac issues

Red flags for hyperaldosteronism

CT of adrenal glands, labetalol (HTN emergency), treat hypokalemia

18 y/o male presents to the ER for HA and visual changes. He also reports N/V. Vitals are stable with the exception of BP 180/112. See rhythm strip. Labs show hypokalemia, elevated plasma aldosterone and low plasma renin. What is your next step?

High PAC, Low PRA, PAC:PRA ratio between 20-40

Classic labs for primary hyperalsoteronism

24 hour urine aldosterone, sodium, creatinine on a high sodium diet (5000 mg), fludrocortisone suppression test, saline suppression test

Secondary workup for primary hyperaldosteronism - confirm those suspicions

High PAC, high PRA, PAC:PRA less than 10

Classic labs for secondary hyperalsoteronism

CT/MRI scan of the adrenals, adrenal vein sampling (determine unilateral vs. bilateral)

Imaging to confirm primary hyperaldosteronism

long term aldosterone receptor antagonist (spironolactone, eplernone)

Treatment plan for hyperaldosteronism caused by bilateral adrenal hyperplasia

normalize serum K and blood pressure

Goals of therapy for hyperaldosteronism caused by bilateral adrenal hyperplasia

laparoscopic complete adrenalectomy, monitor for hyperkalemia, spironolactone OR adenoma resection (HTN is improved)

Treatment plan for hyperalsoteronism caused by unilateral adrenal hyperplasia