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Question 1

adrenal glands
where are they located?
what is it derived from?

Answer 1

suprarenal glands
located above kidney in retroperitoneal

cortex derived from mesoderm
medulla derived from neural crest (is neural tissue)

Question 2

what are the 3 zones of the cortex? what does each produce?

Answer 2

zona glomerulosa: aldosterone?? responsive to angiotensin 2
zona fasciculata: cortisol, androgen?? responsive to ACTH
zona reticularis: cortisol, androgen?? responsive to ACTH

Question 3

what is medulla composed of?

what does it secrete?

Answer 3

composed of post?ganglionic sympathetic cells= chromaffin cells
secrete catecholamines into blood

Question 4

what diseases affect the adrenal cortex? the medulla?

Answer 4

cortex: hyperplasia, atrophy, neoplasms, autoimmune, infections, hemorrhage
medulla: neoplasms

Question 5

what is primary chornic adrenal insufficiency called?

what about inc aldosterone?
inc cortisol?
inc androgen?

Answer 5

addison?? chronic adrenal insufficiency

inc aldosterone: conn syndrome
inc cortisol: cushings
inc androgen: congenital adrenal hyperplasia

Question 6

what causes primary hyperplasia? secondary?
what is most common?
what causes secondary hyperaldosteronism and hypercortisolism?

Answer 6

primary: congenital lack of synthetic enzyme
secondary: inc trophic factor (ACTH, renin/angiotensin)

hyperaldo: hyperplasia of zona glomerulosa bc of inc renin/angiotensin
hypercortisol: hyperplasia zona fasciulata/reticularis due to inc ACTH

Question 7

what is congenital adrenal hyperplasia?

Answer 7

adrenogenital syndrome
lack of a steroid hormone synthetic enzyme
cant produce cortisol/aldosterone so it gets pushed into androgen pathway (you get virilization and salt wasting)

Question 8

what causes adrenal cortical atrophy?

Answer 8

prolongd medicinal glucocorticoids (dec ACTH) leads to bilateral adrenal cortical atrophy

if therapy abruptly withdrawn, atrophic adrenals cannot secrete adeuqate enough hormones so you get secondary adrenal insufficiency

Question 9

is adenoma of adrenal cortex common?

Answer 9

no
gross?? single, solid, encapsulated, yellow
histo: pale, lipid laden, well differentiated cells

can be functional or non function
if too much cortisol?? cushing syndrome
too much aldo: conn syndrome

Question 10

is carcinoma of the adrenal cortex common?

are theyfunctional?

Answer 10

no
when functional, remaining adrenals are atrophic (inc cortisol, dec ACTH secretion by ant. pituitary??> adrenal cortical atrophy)
80% are functional?? when functional, its cushing syndrome

Question 11

adrenal mets come from which 3 places?

what is it called if mets destroy >90% of adrenal tissue?

Answer 11

lung, breasts, melanoma
path: glands enlarged, metastatic tissue present, malignant cells
if destroys 90% tissue??> primary chronic adrenal insufficiency: addisons disesase

Question 12

autoimmune adrenalitis

Answer 12

anti?adrenal Ab
can lead to atrophy
early on: lymphoid infiltrates into cortex
chronic: fibrosis of cortex, medulla is sapred

clinical: addisons disease (due to autoimmune destruction)

Question 13

tuberculosis adrenalitis

Answer 13

underveloped countries, worldwide is most common cause of primary chronic adrenal insufficiency

gland enlarges then atrophies, you see granulomas (macrophages and multinucleate giant cells)

if TB destroys adrenal gland??> addisons disease

Question 14

adrenal hemorrhage

3 etiologies

Answer 14

anti?coag therapy
DIC
bilateral hemorrhage?>acute hemorrhagic necrosis
bacterial sepsis: waterhouse?friderichsen syndrome: caused by neisseria meningiditis and then psueodomonas
septicemia ??> hypotension, DIC??> massive adrenal hemorrhage, skin purpura??> death

Question 15

endocrine causes of adrenal problems

Answer 15

dec trophic hormones lead to secondary adrenal insufficiency
(dec CRH from hypothalamic destruction and dec ACTH from pituitary descturion

inc of each can lead to secondary adrenal hyperplasia (cushings)

Question 16

define cushings

Answer 16

excess cortisol from either:
medicinal glucocorticoids
pituitary corticotrope adenoma (inc ATCH)
adrenal cortical hyperplasia, adneoma, carcinoma
or ectopic ACTH

Question 17

adrenal medulla

which neoplasms are here?

Answer 17

neuroblastoma and
pheochromocytoma: chromaffin cells which secrete catecholamines??>hypertension
rule of 10%? 10% bilateral, malignant, occur in children, extra?adrenal
surgically correctable form of hypertension

Question 18

where is aldosterone (and other mineralcorticoids) produced?
targets?
hormonal effects?
stimulatory factors?

Answer 18

zona glomerulosa
kidneys, blood vessels, heart
na/k regulation, BP regulation
sitmulated by angiotensin 2, high K+, low Na+

Question 19

cortisol/glucocorticoids: where is it produced?
targets?
hormonal effects?
stimulatory factors?

Answer 19

zona fasciculata
targets most cells
protein, fat, carb metabolism
ACTH is stimulatory factor

Question 20

androgens (DHEA, androstenedione): where is it produced?
targets?
hormonal effects?
stimulatory factors?

Answer 20

zona reticularis
targets most cells
sexual maturation and bone/muscle growth
ACTH stimulates it

Question 21

where are catecholamines producd (DA, NE, EPI)
targets?
hormonal effects?
stimulatory factors?

Answer 21

medulla
targets most cells
cardiac stimulation, BP increase
sympathetic fibers stimulate it

Question 22

where does aldosterone work?

Answer 22

increases gene transcrpition in principle cells of the renal collecting duct to reabsorb Na and excrete K

Question 23

how does renin and angiotensin regulate aldosterone?

Answer 23

renin stimulates alpha globulin ??>angiotensin I ??> angiotensin 2, which stimulates adrenal cortex (glomerulosa cells) to produce aldosterone, which leads to renal Na and water retention ??> inc EC fluid volume

Question 24

which factors inhibit aldosterone secretion?

activate it?

Answer 24

inhibited by increasesd EC fluid volume and arterial pressure
activated by dec EC fluid volume and dec arterial pressure and Na+ depletion

Question 25

what 5 things does cortisol effect? | BBIIG?

Answer 25

``` inc blood pressure dec bone formation dec inflammation dec immune function inc gluconeogenesis (and lipoplysis/proteolysis) ``` it is a stress hormone that mobilizes stores for immediate use

Question 26

what does ACTH stimulate to produce cortisol?

Answer 26

stimulates uptake of lipoproteins in fasciculata cells and conversion of cholesterol to pregnenolone (rate limiting step in production of cortisol)

Question 27

what are function of androgens?

Answer 27

female: promote dvlpmnt of pubic and axillary hair contribute to libido males: (less signif after puberty), but testosterone from testicles is major androgen)

Question 28

steps of catecholamine synthesis

Answer 28

tyrosine ?> L?DOPA?> DA?> NE?> Epinephrine homovanillic acid is breakdown product of DA VMA, normetanephrine is bkdwn product of NE metanephrine is bkdwn product of epinephrine

Question 29

DA: target receptor, action, physio response NE EPI

Answer 29

DA: D1, vasodilation in heart/kidney/mesentary inc PVR, pulse pressure, MAP NE: alpha, beta 1, intense vasoconstriciton EPI: alpha, beta 2: vasodilation in skeletal muscle, vasoconstriction in skin/viscera, stimulation of cardiac muscle cells physi: neutral on PVR, CO, HR metab effects: fight or flight?? inc glycogenolysis/lipolysis, dec insulin release

Question 30

what labs do you order for adrenal cortex testing?

Answer 30

aldosterone (plasma, in conjunction w/ renin) cortisol (plasma or 24 hrs urine) androgens (DHEA, androstenedione, testosterone, speicifc steroids, DNA studies

Question 31

what labs do you order for adrenal medulla testing? what factors affect catecholamine measurement?

Answer 31

catecholamines (plasma or 24 hr urine) metanephrines (plasma or 24hr urine) stress, anxiety, exciemtent, drugs (many!), withdrawal states?? test under calm, controlled coniditions with discontinued interfering meds if possible

Question 32

what factors will inc aldosterone? | what factors will dec aldosterone?

Answer 32

low na diet, volume depletion/diuretics, upright posture will increase aldo high na diet, volume overload/saline infusion, supine posture will dec aldo

Question 33

which factors affect cortisol measurement?

Answer 33

cortisol is 90% bound to cortisol binding protein factors that inc CBG (estrogen containing OCPs) will increase total measured cortisol, factors that dec CBG (severe liver dysfunction) will dec total measured cortisol neither should affect urinary or salivary free cortisol cortisol is secreted in cricadian pattern?? highest in AM before waking, lowest at night

Question 34

clincal features of adrenal insufficiency: cortisol deficiency: (6) aldosterone def: (5) aldrenal androgen deficiency (1)

Answer 34

cortisol: fatigue, anorexia/weight loss, hypoglycemia, hyponatremia (dilutional), poor tolerance of stress?vascular collapse?? these can all be primary or 2ndary hyperpigmentation (primary only) aldo: hypvolemia, postural hypotension, hyperkalemia, hyponatremia (salt loss), metabolic acidosis androgen: loss of pubic and axillary hair these are only seen in primary AI

Question 35

labs for primary AI | causes?

Answer 35

labs: low cortisol, low aldosterone, high renin and angiotensin, high ACTH and CRH causes: idiopathic (autoimmune), iatrogenic, infxn (TB, histoplasmosis, HIV), infiltrative disease (mets, amyloidosis), hemorrhage, inherited (congenital hpyerplasia, adrenal leukodystrophy)

Question 36

secondary AI: labs, causes

Answer 36

labs: low cortisol, normal aldo, renin, angiotensin, low CRH, low ACTH you wont have hyperkalemia bc you are able to get aldo from another source than ACTH cause: pituitary/hypo tumor (adenoma, craniopharyngioma) iatrogenic (surgery, x ray, chronic high dose corticosteroid therapy) pituitary infarction (sheehans) infxn, granulomatous dz, idiopathic

Question 37

what is rapid ACTH test? what does it tell you?

Answer 37

large dose of synthetic ACTH (cosyntropin) is given in morning (when cortisol is highest), you see if cortisol responds yes: secondary no: primary

Question 38

steps in evaluating adrenal insufficiency

Answer 38

1. baseline plasma cortisol and ACTH | 2. admin cosyntropin, draw plasma cortisol at 30 and 60 min?? if peak >20, normal, if 200: primary insufficiency

Question 39

2 form so congenital adrenal hyperplasia: | what deficiencies, what are the defects?

Answer 39

21?OHase (other one is 11hydroxylase) so progesterone cannot be convertedinto deoxycorticosterone or deoxycortisol you have mineralcorticoid deficiency and HYPOtension, cortisol deficiency, and increased weak adrenal androgens cortisol def: severe: vascular collpase, vomiting, death in infancy partial: variable lack of tolerance due to stress aldo def: sever: polyuria, volume depletion, hyperkalemia in infancy partial: compensated with adequate salt intake adrenal androgen excess: sever: masculinization of external genitalia in female infants, ioxsexual precocity in boys partial: hrsutism (male patterned hair growth) and irregular menses in women

Question 40

whih step in the pathway is stimulated by ACTH? if there is an ACTH deficiency, what will be affected?

Answer 40

step from cholesterol to pregnenolone

Question 41

pathophys of 11?hydoxylase deficiency

Answer 41

cant go from 11?deoxycorticosterone to corticosterone or 11 doexycortisol to cortisol you get increased mineralcorticoids (excess 11?deoxycorticosterone) which causes HYPERtension you get symptoms of cortisol deficiency you get increased weak adrenal androgens

Question 42

which steroids mainly affect mineralocorticoids? | which steroids mainly affect glucocorticoids?

Answer 42

mineral (aldo): fludrocortisone gluco (cortisol): in lecture: hydrocortisone or prednisone also dexamethasone, also methylprednisolone and prednisolone, prednisone need increased doseing during stress usually dont need to replace mineralcorticoid during secondary adrenal insufficiency except in conditions of volume depletion

Question 43

clinical features of cushings syndrome

Answer 43

``` glucocorticoid excess (hypercortisolism) muscle wasting, weakness easy bruising, poor wound healing, osteoporosis, fractures central obseity glucose intolerance psych disturbance ``` mineralocorticoid excess: salt retention, HTN, edema, hypokalemia, metabolic alkalosis androgen excess: excess hair, acne, amenorrhea

Question 44

lab values for cushings due to adrenal tumor due to ACTH producing pituitary tumor? what happens to each after low dose dexamethasone and high dose dexamethasone?

Answer 44

adrenal tumor: high cortisol, low ACTH , low CRH (will not suppress to dexamethasone) pitutiary tumor? cushings: low CRH, high ACTH, high cortisol, WILL suppress to high dose dexamethasone (so eventually responds to feedback, but set point is higher) ectopic ACTH producing tumor: low CRH, high ACTH, high cortisol (will not suppress to dexamethasone) all will have high urine cortisol

Question 45

steps in evaluating cushings:

Answer 45

1. 24 urine free cortisol, lose dose dexamethasone suppression test (if normal its not cushings) 2. plasma ACTH, high dose dexameth suppression ??> if ACTH undetectable, no suppression?? adrenal tumor ??> if ACTH elevated, no suppression?? ectopic ACTH syndrome ??> if ACTH normal or evelated, suppression to (50% baseline??> cushings disease (pituitary)

Question 46

what are management options for cushings?

Answer 46

ACTH secreting pituitary tumor: remove it though transphenoidal approach, bilateral adrenalectomy if you cant remove: blockers of ACTH secretion by tumor or steroid biosynthesis primary adrenal tumor: surgical resection?? sucessful for adenomas, not carcinomas carcinoma: block steroid biosynthesis, adrenolytic drugs ectopic ACTH syndrome remove/ablate primary tumor with surgery, radiation block therapy: block steroid biosynthesis bilateral adrenalectomy

Question 47

what is drug action of cabergoline and pasireotide? drug action of mifepristone? spironlactone? ketoconazole? aminoglutethimide?

Answer 47

decreased ACTH secretion by tumor mifepristone: blocks glucocorticoid receptor spironolactone: blocks mineralcorticoid receptor ketoconazole and aminoglutethimide: inhibit steroidogenesis

Question 48

what are endocrine causes of hypertension?

Answer 48

1. primary essential HTN 2. renin?angiotensin mediated (coarctation of aorta, renin?secreting tumor, renovascular) 3. mineralocorticoid mediated? primary aldosteronism, cushings, CAH, exogenous glucocorticoids, mineralocortioids 4. volume mediated: acromegaly 5. catecholamine mediated; pheochromocytoma, neuroblastoma, acute stress unkonwn mechanisms: hypercalcemia

Question 49

causes of mineralocortoicoid excess

Answer 49

primary hyperaldosteronism: aldo producing adenoma, idiopathic hyperaldosteronism secondary hyperaldosteronism: renal artery stenosis, diuretic use, renin producing tumor apparent mineralocorticoid excess"?? clinical features mimic this"

Question 50

what are features of primary hyperaldosteronism?

Answer 50

sodium retention with plasma volume expansion hypokalemia due to excessive potassium excretion (remember that in order to reabsorb water you need to reabsorb Na so you lose K)

Question 51

what steps do you do to screen for hyperaldosteronism when you have hypertension and hypokalemia?

Answer 51

plasma renin activity and plasma aldosterone concentration both elevated: secondary hyperaldo (diuretic use, renovascular HTN, renin secreting tumor, coarctation of aorta) if PRA low, PAC high?? primary aldosteronism congenital adrenal hyperplasia, cushings investigate for non aldosterone mediated causes

Question 52

what are confirmatory tests for hyperaldosteronism?

Answer 52

failure of renin (and aldo) to suppress in response to volume expansion by saline infusion failure of renin and aldo to increase in response to volume depletion (lasix) also you can look at see if it changes in posture: you SHOULD see inc in aldo when you stand up

Question 53

how can you tell a difference between aldo producing adenoma and idiopathic hyperaldosteronism?

Answer 53

APA: aldo does not increase with standing IHA: aldo may increase with standing can help to distinguish etiology of primary hyperaldosteronism

Question 54

once you have confirmed primary aldosteronism, whats next?

Answer 54

localizing tests: adrenal CT scan if you have normal microndolatriyt, bilateral masses, its probably not APA screen for GRA? if its high prob APA: do adrenal venous sampling (if you find that its an adenoma: do unilerateral adrenalectomy LOOK AT FLOW CHART

Question 55

what is common cause of secondary hyperaldo?

Answer 55

renal artery stenosis

Question 56

what are 4 drug classes that block RAAS?

Answer 56

renin inhibitors ACE inhibitors (enzyme that catalyzes Ang 1??> Ang 2) angiotensin 2 blockers aldo receptor antagonists

Question 57

what is eplerenone?

Answer 57

mineralcorticoid receptor antagonist

Question 58

pheochromocytoma

Answer 58

rare tumor that can cause catecholamine excess, diff to diagnose symptoms during paryxysms headache, sweating, forceful heartbeat, anxiety, tremor, fatigue, N/V symtpoms between paroxysms: inc sweating, cold hands and feet, weight loss, constipation, POSTURAL HYPOTENSION tumor characteristics: 10% of benign tumors are bilateral, 10% originate in sympathetic ganglia outside renal medulla, 10% are malignant LOOK AT FLOW CHART preferred tx is surgical removal, medical tx is combo of alpha blockers then beta blockers given preoperatively

Question 59

if you suspect unilateral adrenal mass, what do you do?

Answer 59

in young person with clear mass and biochemically proven primary hyperaldosteronism?? do surgery (adrenalectomy) in all others, adrenal vein sampling is recommended? helps to determine is aldo excess is unilteral

Question 60

what is tx of choice for aldo producing adneoma (conns syndrome)?

Answer 60

surgery but if not, mineralcorticoid blockign drugs should be used: spironolactone (also blocks androgen and progesterone receptors) eplerenone: expensive but more specific potassium supplementation may be required

Question 61

what history suggests renal arterial stenosis?

Answer 61

worsening HTN with evidence of atherosclerosis or an abdominal bruit you would confirm with renal arteriogram (would demonstrate high gradient of plasma renin activity from venous drainage of ischemic kidney compared to peripheral circulation), renal ultrasound, MRI

Question 62

what is tx for renal arterial stenosis?

Answer 62

surgery or baloon angioplasty to open stenosed renal artery | HTN can be controlled with drugs (ace inhibitors, ARBs, direct renin inhibitors)

Question 63

which lab value is most specific for pheochromocytoma? which one is most sensitive?

Answer 63

specific: elevation of 24 hour urine metanephrines sensitive: elevation of plasma metanephrines (but they can be falsely elevated in stressed or sick patients)

Question 64

what is phenoxybenzamine?

Answer 64

non selective alpha blocker used before surgery | also propanolol to be used before pheochromocytoma surgery too

Question 65

what is screening test for hyperaldosteronism? | confirmatory test?

Answer 65

screening: serum aldosterone: plasma renin ratio confirm: aldosterone after salt loading or saline admin

Question 66

how do you treat primary renal insufficiency?

Answer 66

hydrocortisone and fludrocortisone