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Block 10 Week 2 > Usera > Adrenal Path > Flashcards

Usera > Adrenal Path Flashcards

1
Q

what kind of corticoids are made in the zona glomerulosa?

A

MINERALOcorticoids

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2
Q

which corticoid is a mineralocorticoid?

A

aldosterone

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3
Q

what kind of corticoids are made in the zona fasciculata?

A

GLUCOcorticoids

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4
Q

cortisol is a mineralo or gluco corticoid?

A

glucocorticoid

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5
Q

what is made in the zona reticularis?

A

sex hormones

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6
Q

what is the organization of the zonas in the kidney, from external to internal?

A 
capsule
zona glomerulosa
zona fasciculata
zona reticularis
medulla
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7
Q

from what is the adrenal medulla derived?

A

neural crest

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8
Q

what does the adrenal medulla produce?

A

catecholamines

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9
Q

what are the 3 main kinds of adrenocortical HYPOfxn?

A
  1. acute adrenocortical insuff
  2. Addison’s dz
  3. congenital adrenal hyperplasia
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10
Q

what are the 3 main causes of acute adrenocortical insuff?

A
  1. abrupt withdrawal of corticosteroids
  2. anticoagulation therapy
  3. Waterhouse-Friderichsen syndrome
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11
Q

wtf is waterhouse-friderichsen syndrome?

A

bilateral adrenal hemorrhage d/t severe bacterial infection

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12
Q

what bug is usu assoc w/ waterhouse-friderichsen syndrome?

A

NEISSERIA MENINGITIDIS

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13
Q

if you have DIC w/ waterhouse-friderichsen syndrome, what happens?

A

endotoxic shock

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14
Q

what is another name for Addison’s dz?

A

chronic adrenocortical insuff

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15
Q

T/F: 80% of addison’s dz is autoimmune

A

TRUE

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16
Q

what are the 5 main causes of addison’s dz?

A
  1. autoimmune
  2. infectious
  3. adrenogenital syndrome
  4. mets
  5. AIDS
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17
Q

what are the infectious causes of addison’s dz?

A

TB

histoplasmosis

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18
Q

what 2 types of mets can cause addison’s dz?

A

lung cancer

RCC

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19
Q

what are the 3 physical findings of addison’s dz?

A
  1. weakness
  2. hypotension
  3. hyperpigmentation
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20
Q

why do addison’s pts get hyperpigmentation?

A

d/t increased ACTH

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21
Q

in addison’s dz, what happens to Na, K, & pH?

A

hyponatremia
hyperkalemia
metabolic acidosis

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22
Q

what happens to blood sugar in addison’s dz?

A

fasting hypoglycemia

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23
Q

what happens to WBCs in addison’s dz?

A

lymphocytosis
eosinophilia
neutropenia

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24
Q

what is the metyrapone test?

A

inhibits 11 beta hydroxylase & prevents cortisol synthesis so it SHOULD result in ACTH secretion and increased steroid precursors

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25
Q

what happens to ACTH in addison’s dz?

A

it’s INCREASED

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26
Q

do addison’s dz pts respond to ACTH stimulation tests?

A

nope

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27
Q

what happens in addison’s pts if you do a metyrapone test?

A

ACTH increases
but
there is NO INCREASE in 11-deoxycortisol

this suggests adrenal insuff

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28
Q

what is another name for adrenogenital syndrome?

A

congenital adrenal hyperplasia

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29
Q

what is congenital adrenal hyperplasia?

A

autosomal recessive disorder of adrenal biosynthetic enzymes

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30
Q

which enzyme is most often deficient in congenital adrenal hyperplasia?

A

21-hydroxylase (90-95% of cases)

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31
Q

what are the 3 different types of enzyme deficiencies in congenital adrenal hyperplasia?

A
  1. 21-hydroxylase def (most common)
  2. 11-hydroxylase
  3. 17-hydroxylase def
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32
Q

what is 17-hydroxylase def assoc w/?

A

hypogonadism

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33
Q

what happens to ACTH in congenital adrenal hyperplasia?

A

increases

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34
Q

what happens to the enzyme precursors in congenital adrenal hyperplasia?

A

the precursors proximal to the enzyme block will accumulate
increased 17-ketosteroids
testosterone

35
Q

why do newborn girls with congenital adrenal hyperplasia have ambiguous genitalia?

A

because of increased androgen production

36
Q

if a baby girl comes out with ambiguous genitalia, what do you do?

A

CHROMOSOMAL ANALYSIS

37
Q

how is puberty affected in congenital adrenal hyperplasia?

A

precocious puberty (in M & F)

38
Q

what is the growth pattern of children with congenital adrenal hyperplasia?

A

rapid growth as children

39
Q

what do adults w/ congenital adrenal hyperplasia look like?

A

short stature

40
Q

which 2 deficiencies of congenital adrenal hyperplasia result in ambiguous genitalia &/or weird growth?

A

21 & 11 hydroxylases

41
Q

which hydroxylase deficiency of congenital adrenal hyperplasia results in DECREASED glucocorticoids & androgens?

A

17-hydroxylase deficiency

42
Q

what happens to males & females with 17-hydroxylase deficiency?

A

F: delayed menarche & secondary sexual characteristics
M: pseudohermaphroditism

43
Q

what 3 physical findings are involved in a workup for ambiguous genitalia?

A
  1. clitoris hypertrophy
  2. poor development of labial structure
  3. no palpable gonad
44
Q

what are the 2 types of adrenocortical hyperfxn?

A
  1. Cushing’s syndrome

2. Hyperaldosteronism

45
Q

what is the diff btwn cushing’s syndrome & dz?

A 
SYNDROME = high cortisol & the assoc sx
DZ = high ACTH, which RESULTS in high cortisol
46
Q

what are 4 possible causes of Cushing’s syndrome?

A
  1. corticosteroid use
  2. cushing’s dz
  3. hyperfxnal adrenal adenoma
  4. ectopic ACTH production
47
Q

what is Conn’s syndrome?

A

hyperaldosteronism d/t hyperfxnal adrenal adenoma

48
Q

what are the 6 sx assoc w/ Cushing’s syndrome?

A
  1. hirsutism
  2. rounded face
  3. hump
  4. easy bruising
  5. abdominal weight gain
  6. abdominal striae
49
Q

why do Cushing’s pts gain weight?

A

hyperinsulinism secondary to hyperglycemia

50
Q

where does fat deposit in Cushing’s pts?

A

face
nape (hump)
trunk

51
Q

why do Cushing’s pts get hirsutism?

A

increased androgens

52
Q

why do Cushing’s pts get abdominal striae?

A

weakened collagen + BV rupture

53
Q

why do Cushing’s pts have diastolic HTN?

A

increased mineralocorticoids

54
Q

why do Cushing’s pts have muscle weakness?

A

muscle breakdown for gluconeogenesis

55
Q

what will you find on a urine test of a Cushing’s pt?

A

increased urine free cortisol

56
Q

what happens if you give a Cushing pt a dexamethasone suppression test?

A

low-dose cannot suppress cortisol

high-dose can suppress in cushing DZ ONLY (not syndromes!)

57
Q

what happens to pH in Cushing pts?

A

hypokalemic metabolic alkalosis

58
Q

what happens to Cushing pts’ blood sugar?

A

hyperglycemia

59
Q

what zone might an ALDOSTERONE-secreting adrenal adenoma be found in?

A

zona glomerulosa

60
Q

what are the 2 clinical findings in Conn’s syndrome?

A

diastolic HTN

muscle weakness

61
Q

what are the 4 lab findings in Conn’s syndrome?

A

hypernatremia
hypokalemia
alkalosis
decreased plasma renin

62
Q

what are the 2 types of adrenal MEDULLARY hyperfxn?

A
  1. pheochromocytoma

2. neuroblastoma

63
Q

what is a pheochromocytoma?

A

catecholamine-secreting tumor of the adrenal medulla (epi & NE)

64
Q

what is the rule of 10s for pheo?

A

10% bilateral
10% malignant
10% extramedullary

65
Q

what is a paraganglioma?

A

a manifestation of pheo which only produces NE

no phenylethamin-N-methyltransferase

66
Q

what is pheo assoc w/?

A

NF-1
MEN IIa & IIb
von hippel lindau

67
Q

what are the physical findings of pheo related to?

A

increased sympathetic tone

68
Q

what are the 7 physical findings of pheo?

A
  1. paroxysmal &/or sustained systolic HTN w/ palpitations
  2. HA
  3. sweating
  4. anxiety
  5. chest pain
  6. orthostatic intolerance
  7. constipation
69
Q

what is the best CONFIRMATORY test for pheo?

A

plasma free metanephrines

70
Q

what is the best SENSITIVITY test for pheo?

A

24 hour urine metanephrines

71
Q

how do pheo pts react to clonidine?

A

no suppression

72
Q

what is the blood sugar of pheo pts like?

A

hyperglycemia

73
Q

how can you stabilize a pheo pt pre-operatively?

A

alpha inhibitor & beta blocker

you HAVE to do this

74
Q

what is neuroblastoma?

A

malignant neoplasm of post-ganglionic sympathetic neurons

75
Q

what age group is commonly affected by neuroblastoma?

A

<5 yo

76
Q

what is the marker of aggressive behavior in neuroblastoma?

A

N-MYC amplification

77
Q

what % of neuroblastoma cases have N-MYC amplification?

A

20-30%

78
Q

what % of neuroblastomas are metastatic at dx?

A

70% :(

79
Q

what group has a GOOD prognosis in neuroblastoma?

A

<1 yo

hyperdiploid

80
Q

what group has a BAD prognosis in neuroblastoma?

A

> 1 yo

1p deletion

81
Q

how does neuroblastoma look on histo?

A

small round blue cell tumor

82
Q

how does neuroblastoma look on physical exam?

A

large, palpable abdominal mass

83
Q

what happens to the urine of neuroblastoma pts?

A

increased urine VMA & HVA

84
Q

what is the overall survival w/ neuroblastoma & what does it depend on?

A

40%

depends heavily on prognostic factors

Block 10 Week 2 (6 decks)

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