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Endo > Adrenal Insufficiency and Congenital Adrenal Hyperplasia > Flashcards

Adrenal Insufficiency and Congenital Adrenal Hyperplasia Flashcards

1
Q

what is the precursor for steroid hormone synthesis

A
  • lipid
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2
Q

zona glomerulosa is responsive to _____

A
  • AGII
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3
Q

zona fasciculata is responsive to ____

A
  • ACTH
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4
Q

what diseases affect the adrenal cortex

what diseases affect the adrenal medulla

A
  • atrophy
  • hyperplasia
  • neoplasms
  • autoimmune
  • infections
  • hemorrhage
  • neoplasm
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5
Q

cortex is derived from

A
  • mesoderm
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6
Q

medulla is derived from

A
  • neural crest
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7
Q

ACTH is derived from

A
  • POMC (proopiomelanocortin)
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8
Q

further breakdown of ACTH into

what does this do

A
  • alpha melanocyte stimulating hormone

- stimulate pigmentation in the skin

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9
Q

ACTh works through which secondary messengers

A
  • cAMP

- PKA

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10
Q

what enzyme in the adrenal cortex is stimulated by AG II

A
  • aldosterone synthase
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11
Q

is aldosterone production regulated by ACTH

what is it regulated by

A
  • no

- renin-angiotensin

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12
Q

effects of excess cortisol on the brain

A
  • depression

- psychosis

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13
Q

effects of excess cortisol on adipose tissue distribution

A
  • promotes visceral obesity
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14
Q

effects of excess cortisol on endocrine system

A
  • decrease LH, FSH, TSH, and GH secretion
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15
Q

the problem in primary adrenal insufficiency is where

levels of hormones expected:
CRH
ACTH
cortisol
aldosterone
RAAS system
A
  • adrenal cortex
levels of hormones expected:
CRH: elevated
ACTH: elevated
cortisol : low
aldosterone: low
RAAS system : elevated
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16
Q

most common cause of primary adrenal insufficiency in developed countries

in developing countries (which ones)

A
  • autoimmune (Addison’s disease)

- infections (TB, histoplasmosis, HIV)

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17
Q

infiltrative causes of primary adrenal insufficiency

A
  • metastasis

- amyloidosis, hemochromatosis, sarcoidosis

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18
Q

hemorrhage causes of primary adrenal insufficiency

A
  • disseminated infections (N. meningitidis) - Waterhouse Friderichsen Syndrome
  • anticoagulation
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19
Q

drugs that can cause primary adrenal insufficiency

A
  • ketoconazole

- rifampin

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20
Q

how congenital hyperplasias cause primary adrenal insufficiency

A
  • impair cortisol and aldosterone synthesis
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21
Q

how adrenal leukosdystrophy causes primary adrenal insufficiency

A
  • impair cholesterol transport into adrenal gland
22
Q

what happens in Addison’s disease

what is spared

A
  • atrophy of adrenal gland after exposure to destructive antibodies
  • the medulla is spared
23
Q

what happens in Waterhouse Friderichsen syndrome

A
  • septic due to N. meningitidis
  • hypotension with DIC (purpura)
  • adrenal hemorrhage
24
Q

features of primary adrenal insufficiency due to elevated ACTH

A
  • hyperpigmentation of skin/mucosa
25
Q

features of primary/secondary adrenal insufficiency due to cortisol deficiency

A
  • hypotension
  • abdominal pain
  • hyponatremia
  • hypoglycemia
26
Q

features of primary adrenal insufficiency due to aldosterone deficiency

A
  • hypovolemia
  • salt craving (hyponatremia)
  • hyperkalemia
  • metabolic acidosis
27
Q

features of primary adrenal insufficiency due to adrenal androgen deficiency

A
  • decreased pubic and axillary hair
28
Q

hyponatremia in primary adrenal insufficiency due to _____ and ____ deficiency

A
  • cortisol

- aldosterone

29
Q

secondary adrenal insufficiency - the problem is where

levels of hormones expected:
CRH
ACTH
cortisol
aldosterone
RAAS system
A
  • hypothalamus and/or pituitary
levels of hormones expected:
CRH: low
ACTH: low
cortisol: low
aldosterone: normal 
RAAS system: normal
30
Q

causes of secondary adrenal insufficiency

A
  • congenital hypopituitarism
  • pituitary/hypothalamic tumors
  • pituitary infarct
31
Q

what drugs can cause secondary adrenal insufficiency (this is the most common cause of secondary adrenal insufficiency)

how

A
  • exogenous glucocorticoids
  • decreases ACTH through negative feedback
  • bilateral adrenal cortical atrophy
32
Q

what granulomatous diseases can cause secondary adrenal insufficiency

A
  • sarcoidosis

- histiocytosis

33
Q

hyponatremia in secondary adrenal insuffiency is due to ________ deficiency

A
  • ONLY cortisol
34
Q

how do we diagnose adrenal insufficiency

how does it work

A
  • Cosyntropin stimulation test
  • give ACTH
  • measure cortisol levels
35
Q

in what case would a patient with true secondary adrenal insufficiency have a falsely low normal cortisol response to ACTH

A
  • in acute atrophy of adrenals

- adrenals take time to fully atrophy

36
Q

what is the peak cortisol concentration that distinguishes between normal adrenal function and adrenal insufficiency

A
  • 18 mcg/dl
37
Q

when can you diagnose adrenal insufficiency without a cosyntropin stimulation test

A
  • when cortisol is CLEARLY low

- and ACTH is CLEARLY high

38
Q

treatment of primary adrenal insufficiency

what meds

A
  • glucocorticoid replacement (hydrocortisone or prednisone)
  • mineralocorticoid replacement (hydrocortisone or fludrocortisone)

WE GENERALLY USE HYDROCORTISONE IV FOR BOTH

39
Q

importance of dosing medications for adrenal insufficiency during stress

A
  • increase dosing
40
Q

treatment of secondary adrenal insufficiency

what meds

A
  • glucocorticoid replacement (hydrocortisone or prednisone)
41
Q

all potencies of glucocorticoids are relative to

potency of prednisolone
potency of prednisone
potency of dexamethasone

A
  • hydrocortisone
  • 4.0
  • 3.5
  • 30
42
Q

what is the physiologic dose of glucocorticoids per day

patients using higher than this are at risk for what when steroids are withdrawn

why?

A
  • 20 mg/day
  • secondary adrenal insufficiency
  • you’re giving the med so you have decreased feedback on ACTH
  • decreased ACTH causes pituitary atrophy
43
Q

what from the kidney can stimulate the release of vasopressin

A
  • AG II
44
Q

inheritance of congenital adrenal hyperplasia

A
  • autosomal recessive
45
Q

what is the cause of congenital adrenal hyperplasia

what leads to the hyperplasia

A
  • deficiency of enzyme is steroid synthesis pathway

- chronically high ACTH production leads to hyper plastic adrenal glands

46
Q

most common enzyme missing in congenital adrenal hyperplasia

A
  • 21-hydroxylase
47
Q

21-hydroxylase deficiency

  • aldosterone levels:
  • cortisol levels:
  • DHEA levels:
  • androstenedione levels:

symptoms

treatment

A
  • aldosterone levels: low
  • polyuria, volume depletion, hyperkalemia
  • cortisol levels: low
  • vascular collapse, vomiting, death in infancy
  • DHEA levels: high
  • androstenedione levels: high
  • masculinization of external genitalia in females
  • precocious puberty in boys
  • glucocorticoid and mineralocorticoid replacement
  • glucocorticoid to suppress ACTH
48
Q

11-hydroxylase deficiency

  • aldosterone levels:
  • cortisol levels:
  • DHEA levels:
  • androstenedione levels:

important symptoms

treatment

A
  • aldosterone levels: low
  • cortisol levels: low
  • DHEA levels: high
  • androstenedione levels: high
  • hypertension due to high 11-deoxycorticosterone
  • glucocorticoid to suppress ACTH
  • antihypertensives
49
Q

17-hydroxylase deficiency

  • aldosterone levels:
  • cortisol levels:
  • DHEA levels:
  • androstenedione levels

why is BP not low

A
  • aldosterone levels: high
  • cortisol levels: low
  • DHEA levels: low
  • androstenedione levels: low
  • lack of secondary sex characteristics
  • due to excess mineralocorticoids
50
Q

importance of 11-deoxycorticosterone in excess

A
  • promotes fluid and water retention

- suppressed plasma renin and aldosterone secretion

51
Q

treatment of 17-hydroxylase deficiency

A
  • exogenous hydrocortisone (or other glucocorticoid) to suppressed ACTH production
  • induce menses with oral estrogen and progesterone

Endo (10 decks)

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