disorders of the adrenal glands Flashcards

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1
Q

List the 3 sections of the adrenal cortex and what each produces

A
  1. zona glomerulosa: mineralocorticoids: aldosterone
  2. zona fasciculata: glucocorticoids: cortisol
  3. zone reticularis: adrenal androgens: mostly DHEA
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2
Q

cortisol is excreted in the urine in what form

A

17 hydroxysteroid (17OHS)

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3
Q

androgen is excreted in the urine in what form

A

17 ketosteroids (17KS)

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4
Q

what is the sign and symptom complex resulting from prolonged expsore to glucocorticoid hormones

A

cushings syndrome

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5
Q

List the causes of ACTH-dependent cushing’s syndrome

A
  • excessive ACTH production from pituitary (pituitary adenoma): most common cause
  • ectopic ACTH producing tumor
    • small cell lung CA is most common
  • excessive CRH production from hypothalamus
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6
Q

List the causes of ACTH-independent cushing’s syndrome

A
  • adrenal tumor
  • neuroendocrine tumors
  • pheochromocytoma
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7
Q

which type of cushing’s syndrome, ACTH dependent or independent, is more common? Is it more likely in males or females

A
  • ACTH dependent: 70%
  • 8-10x more likely in females
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8
Q

clinical presentation

  • weight gain/obesity
    • facial plethora “moon face”
    • truncal obesity with extremity wasting
    • buffalo hump
  • fatigability and weakness
    • mostly proximal muscles
  • abdominal straie
  • acne and abnormal hair growth
  • edema
  • HTN
  • insulin resistance
  • vertebral fx, hypercalcuria, kidney stones
  • dysphora, depression, paranoia
A

cushing’s syndrome

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9
Q

What two hormone levels do you get to work up possible cushing’s syndrome

A
  • plama cortisol level
    • 8AM
    • 4PM
  • serum ACTH level
    • 8AM
    • 4PM
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10
Q

what is an abnormal result for plasma cortisol level

A
  • > or = 25 ug/dl at 8 AM
  • less than the anticipated drop of 1/3 to 2/3 at the 4PM reading
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11
Q

what is abnormal serum ACTH levels

A
  • > 80 pg/ml at 8am; >50 pg/ml at 4pm
  • < 20 pg/ml at any time
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12
Q

Are cortisol and ACTH levels expected to be high or low in ACTH independent

A
  • cortisol: high
  • ACTH: low
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13
Q

Are cortisol and ACTH levels expected to be high or low in ACTH dependent

A
  • cortisol: high
  • ACTH: high
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14
Q

Why is 11-deoxycortisol measured at 8am when working up for cushing’s syndrome

A
  • 11-deoxycortisol is a cortisol precursor
  • it should be converted to cortisol and be undetectable at 8am
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15
Q

What is the gold standard for screening for cushing’s syndrome? What is an abnormal result

A
  • Dexamethasone suppression test (DST): given at night, expect ACTH and cortisol levels to be low the next morning
  • abn result: cortisol > 5 ug/d is highly suspicious
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16
Q

What is the main stay for diagnosis for cushing’s syndrome? What is an abnormal result

A
  • 24 hour urine free cortisol
  • measures 17-OCHS
  • abnormal: cortisol > 125 ug/d is indicative of cushings
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17
Q

describe salivary sampling used to work up cushing’s syndrome or adrenal insufficiency

A
  • free cortisol diffuses freely into saliva
  • sample saliva obtained after rinsing mouth but before brushing teeth at
    • 9:00 am 3 mornings in a row when evaluating for adrenal insufficiency
    • 11:00pm 3 nights in a row when evaluating for cushing’s syndrome
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18
Q

abnormal results for salivary sampling in cushing’s syndrome vs adrenal insufficiency

A
  • cushing’s syndrome: late evening salivary cortisol elevated
  • adrenal insufficiency: morning salivary cortisol low
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19
Q

MOA of Mitotane

A
  • induces permanent destruction of the adrenocortical cells
  • AKA medical adrenalectomy
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20
Q

MOA of Ketoconazole in cushing’s syndrome? major side effect?

A
  • antifungal with cortisol-reducing effects in higher doses
  • liver toxicity
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21
Q

MOA of Metyrapone in cushing’s syndrome?

A
  • inhibits cortisol synthesis
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22
Q

What is the most common cause of cushing’s syndrome

A

ACTH producing pituitary adenoma

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23
Q

What is the most common cause of cushingoid symptoms

A

exogenous steroid use

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24
Q

what is the inadequate production of mineralocorticoids, glucocorticoids, and or sex androgens

A
  • adrenal insufficiency
    • when all three are present -> addison’s disease
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25
Q

what is the most common cause of primary adrenal insufficiency

A

addison’s disease: autoimmune destruction of adrenal gland

26
Q

what are some infectious agents that can cause adrenal insufficiency

A
  • histoplasmosis
  • TB
  • coccidiodomycosis
27
Q

What is the most common cause of secondary adrenal insufficiency

A
  • suppression of HPA axis through abrupt cessation of exogenous steroid
28
Q

list drugs that reduce corticosteroids and can cause secondary adrenal insufficiency

A
  • phenytoin
  • opiates
  • ketoconazole
  • rifampin
29
Q

list the hallmark tetrad of adrenal insufficiency

A
  • weakness/fatigue
  • weight loss/anorexia
  • hyperpigmentation
  • hypotension (orthostatic)
30
Q

clinical presentation suggest deficiency in which hormone

  • weakness
  • fatigue
  • hypoglycemia
  • weight loss
  • anorexia
  • nausea
  • vomiting
  • abd pain
A

glucocorticoid deficiency

31
Q

clinical presentation suggest deficiency in which hormone

  • Na++ wasting
    • hyponatremia, salt craving
  • hypovolemia
  • orthostatic hypotension
  • hyperkalemia
  • mild metabolic acidosis
A

Mineralocorticoid deficiency

32
Q

clinical presentation suggest deficiency in which hormone

  • loss of axillary and pubic hair in females
  • amenorrhea
A

adrenal androgen deficiency

33
Q

What is an abnormal result in the plasma cortisol level in the work up for adrenal insufficiency

A
  • < 5 ug/dl in the AM
34
Q

What is an abnormal result in the serum ACTH level in the work up for adrenal insufficiency

A
  • > 80 at 8am; > 50 at 4 pm
  • < 20 at any time
35
Q

what do you expect cortisol and ACTH levels to be in primary and secondary adrenal insufficiency

A
  • primary (addison’s disease: cortisol low, ACTH high
  • secondary: corticol low, ACTH low
36
Q

explain the Cosyntropin stimulation test

A
  • evaluates the ability of the adrenal gland to respond to ACTH administration: gold standard for diagnosis
  • procedure
    • obtain baseline cortisol level
    • administer bolus of ACTH
    • measure cortisol at 30 min and 60 min
  • abormal result: cortisol level fails to increase > 18 mcg/dl
37
Q

What is the benefit of the Metyrapone stimulation test

A
  • can be completed while on glucocorticoid supplementation
38
Q

what is an abnormal response on the Metyrapone stimulation test in regards to 24 hour 17-OCHs excretion, serum cortisol, plasma ACTH, and serum 11-deoxycortisol

A
  • 24 hour 17-OCHs excretion: less than doubling from baseline
  • serum cortisol: > 5
  • plasma ACTH: < 75
  • serum 11-deoxycortisol: < 7
39
Q

Management of glucocorticoid deficiency

A
  • hydrocortisone
  • prednisone
  • dexamethasone
40
Q

Management of mineralcorticoid deficiency

A

fludrocortisone

41
Q

Management of adrenal androgen deficiency

A
  • DHEA
42
Q

What should be done to the dose of glucocorticoids in the face of highly stressful events

A

increase dose

43
Q

Two causes of primary hyperaldosteronism (Conn’s syndrome)

A
  • adrenalcorticol adenoma
  • cortical hyperplasia
44
Q

elevated plasma and urine aldosterone, low plasma renin levels are indicative of

A

primary hyperaldosteronism (Conn’s syndrome)

45
Q

management of primary hyperaldosteronism (Conn’s syndrome)

A
  • surgical removal of adenoma
  • medical = spironolactone and antihypertensive agents
46
Q

clinical presentation

  • HTN
  • hypokalemia
  • muscle weakness
  • paresthesias
  • HA
  • polyuria and polydipsia
A

primary hyperaldosteronism (Conn’s syndrome)

47
Q

What is a pheochromocytoma

A
  • rare, catecholamine-producing tumor of neurochromaffin cells
  • 10-15% malignant
  • fatal if undiagnosed and untreated
48
Q

extra-adrenal pheochromocytomas are called

A

paragangliomas

49
Q

majority of pheochromocytomas arise from

A

adrenal medulla

50
Q

pheochromocytomas can also occur in what autosomal dominant hereditary syndromes

A
  • MEN2
  • von hippel-lindau syndrome
  • neurofibromatosis type 1
51
Q

pheochromocytomas is characterized by classic paroxysm attacks of

A
  • HA
  • sweating
  • palpitations
  • profound acute HTN at the time of episode
    • usually last 30-40 min
    • may be precipitated by displacement of abdominal contents
    • tend to increase in frequency and severity over time
52
Q

what test is used to definitively diagnose pheochromocytomas

A
  • clonidine suppression test
53
Q

clonidine suppression test procedure

A
  • stop hypotensive meds x 24 hours, fast overnight
  • baseline BP, pulse and labs taken
  • administer clonidine
  • continue to monitor BP, pulse, and labs
  • results:
    • expect suppression of NE and epi
54
Q

tx for pheochromocytoma

A
  • IV nitroprusside for crisis
  • chemical sympathectomy: pure alpha blocker, then beta blocker
  • surgical excision is definitive tx
55
Q

what is the rule of 10s in Pheochromocytoma

A
  • 10% w/o HTN
  • 10% extraadrenal
  • 10% extraabdominal
  • 10% children
  • 10% familial
  • 10% bilateral
  • 10% metastatic disease at diagnosis
56
Q

after the initial workup for pheochromocytomas, what should be done

A
  • anatomic localization
    • CT, MRI, PET, MIBG scan, OctreoScan
57
Q

what is a adrenal incidentaloma

A
  • mass lesion greater than 1 cm discovered incidentally by radiologic exam
  • prevalence higher in obese, HTN, and DM patients
  • 85-90% are non-functional
58
Q

an adrenal incidentaloma that is lipid rich is more indicative of a

A

benign lesion

59
Q

evaluation of hormonal secretion after finding a adrenal incidentaloma

A
  • plasma cortisol, serum ATCH, serum DHEA, plasma aldosterone
  • sxs of cushing?
    • yes: 24 hr urinary free cortisol
    • no: 1 mg overnight dexamethasone suppression
  • suspicion for pheo?
    • 24 hr urine metanephrines, catecholamines
60
Q

when is a fine needle aspiration indicated in adrenal incidentaloma

A
  • if primary malignancy elsewhere
  • dont use if
    • evidence of pheo
    • known widespread metastatic dz
61
Q

when is resection indicated for adrenal incidentaloma

A
  • > 2 cm