CUSHING SYNDROME AND CUSHING DISEASE Flashcards

1
Q

what hormone does the zona glomerulosa release?

A

mineralcorticoids: aldosterone

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2
Q

what hormone does the zona fasciculata release?

A

glucocorticoids: cortisol, corticosterone, cortisone

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3
Q

what hormone does the zona reticularis stimulate?

A

androgens that stimulate masculinization-> dehydroepiandrosterone

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4
Q

what hormone does the adrenal medulla secrete?

A

stress hormones that stimulate the sympathetic ANS: epinephrine and norepinephrine

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5
Q

what does the hypothalamus do in the HPA axis?

A

it secreted CRH that stimulates the release of ACTH from the anterior pituitary gland

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6
Q

when ACTH is released in the HPA axis, what does ACTH do next?

A

like CRH, ACTH follows the circadian rhythm

ACTH increases in the early morning hours before awakening with peak levels in the morning around 8:00 and decrease in the evening

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7
Q

what does the adrenal gland (cortex) do in the HPA axis?

what things are secreted from adrenal gland-only two

A

the zona fasciculata (cortisol) and zona reticularis (androgens) are stimulated by ACTH

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8
Q

what is the zona glomerulosa (aldosterone) stimulated by?

A

it is primarily regulated by the renin-angiotensin system and potassium levels

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9
Q

what is cushing syndrome?

A

a constellation of clinical abnormalities caused by chronic high blood levels of cortisol

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10
Q

what is primary hypercortisolism?

A

increased production of glucocorticoids by adrenal glands
tumor- adrenal adenoma or adrenal carcinoma

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11
Q

what is secondary hypercortisolism?

A
  1. cushings disease which is a subtype of cushings syndrome
    • increased pituitary ACTH production leading to
      adrenal gland hyperplasia (most likely d/t pituitary
      adenoma)
  2. ectopic ACTH syndrome
    • increased ACTH production outside the pituitary and
      adrenal glands (paraneoplastic syndromes- SMALL
      CELL LUNG CANCER )
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12
Q

are primary and secondary hypercortisolism endogenous or exogenous?

A

endogenous

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13
Q

is endogenous or exogenous the most common form of hypercortisolism and what is the reason?

A

exogenous- medical use of glucocorticoids

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14
Q

what does exogenous cortisol cause at the level of the hypothalamus and anterior pituitary?

A

a negative feedback that leads to decreased CRH and ACTH

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15
Q

what occurs during long-term exogenous glucocorticoid use?

A
  1. cortisol will not be released by the body
  2. atrophy of the zona fasciculata
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16
Q

what occurs when a patient abruptly discontinues exogenous glucocorticoid use?

A

the body cannot produce enough cortisol and adrenal insufficiency will occur leading to an adrenal crisis

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17
Q

what is ACTH independent cushings syndrome?

A

the adrenal glands produce cortisol without stimulation from ACTH

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18
Q

what is ACTH dependent cushings syndrome?

A

the body makes too much ACTH which in turn increases the production of cortisol

19
Q

what is the clinical presentation of cushings?

A

moon face
truncal (central) obesity
prominent supraclavicular and dorsal cervical fat pads (buffalo hump)
thin distal extremities and fingers
proximal muscle wasting
recurrent opportunistic or bacterial infections such as crypto neoformans, or candida species
thin and atrophic skin
violaceous striae wider than 1 cm (abdomen)

20
Q

does cushings syndrome cause hypo or hypertension?

A

atrial hypertension

21
Q

what does the increased blood pressure in cushings syndrome lead to?

A

pseudohyperaldosteronism

22
Q

what are the different diagnostic tests for high cortisol/ cushings?

A
  1. 24-hr urine for free cortisol level
  2. dexamethasone suppression test
  3. plasma ACTH levels
  4. CRH stimulation test
  5. serum or saliva cortisol measurement at midnight to assess the normal fall of cortisol
23
Q

what high cortisol result will you seen in a 24-hr urine for free cortisol level ?

A

> 4 times the upper limit of normal cortisol levels

24
Q

what is the dexamethasone suppression test?

A
  1. 1-2 mg of dexamethasone is given orally at 11 pm (normally should decrease ACTH) and serum cortisol is measured at 8 am the next morning (normally should decrease serum cortisol)
  2. increase cortisol levels= positive test and further testing is needed
25
Q

what is the plasma ACTH level test?

A

it is measured to determine the cause of cushing syndrome

26
Q

what is the CRH stimulation test?

A

patient is given synthetic CRH

27
Q

what does an increase in ACTH and cortisol during the CRH stimulation test mean, and what are the next steps?

A
  1. cushings disease
  2. order an MRI of the brain w/ contrast
28
Q

what does no increase in ACTH and cortisol during the CRH stimulation test mean, and what are the next steps?

A
  1. ectopic ACTH secretion
  2. order a CT scan of the chest, abdomen, and pelvis
29
Q

what if there is an increase in levels during the plasma ACTH level test?

A
  1. high levels -> pituitary (cushings disease or ectopic source)
30
Q

what if there is a decrease in levels during the plasma ACTH level test?

A
  1. low levels -> primary adrenal cause (adenoma, carcinoma, exogenous glucocorticoids)
31
Q

what is the first diagnostic thing you should do before testing?

A

exclude exogenous glucocorticoid use

32
Q

what are the treatment options/types for hypercortisolism?

A
  1. stopping exogenous glucocorticoids- gradual
    withdrawal
  2. pituitary, adrenal, or ectopic ACTH-producing tumor
    treatments
  3. adrenalectomy
  4. nonresectable tumor treatments
33
Q

what is the treatment for pituitary, adrenal, or ectopic ACTH-producing tumors?

A
  1. surgical removal
  2. radiation
34
Q

what will patients with an adrenalectomy require?

A

glucocorticoid replacement therapy

35
Q

what are the treatments for nonresectable tumors?

A
  1. glucocorticoid receptor antagonists
  2. adrenal enzyme inhibitors
  3. dopamine agonists
36
Q

what is an example of a glucocorticoid-receptor antagonist and its MOA?

A

mifepristone- blocks effects of cortisol in tissues

37
Q

what is an example of a adrenal enzyme inhibitors and its MOA?

A

ketoconazole and metopirone- decrease cortisol production

38
Q

what is the MOA of a dopamine agonist?

A

inhibits ACTH secretion

39
Q

what is a pheochromocytoma?

A

a catecholamine-secreting tumor that mimics over-activity of the sympathetic nervous system

40
Q

where do the majority (90%) of pheochromocytomas originate?

A

in the adrenal medulla where catechomaines are

41
Q

what is the BIG 3 clinical presentation of pheochromocytoma?

A
  1. HA
  2. tachycardia/palpitations
  3. sweating
42
Q

what are the non- big 3 clinical presentations of pheochromocytoma?

A
  1. HTN (sustained or paroxysmal)- most common symptom
  2. elevated metabolic rate
  3. hyperglycemia
  4. nervousness
43
Q

what is the most common symptom of pheochromocytoma?

A

HTN (sustained or paroxysmal)

44
Q

what is the treatment of pheochromocytoma?

A

surgical resection