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Block 10 Week 2 > Usera > Adrenal Path > Flashcards

Usera > Adrenal Path Flashcards

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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
what happens to ACTH in addison's dz?
it's INCREASED
26
do addison's dz pts respond to ACTH stimulation tests?
nope
27
what happens in addison's pts if you do a metyrapone test?
ACTH increases but there is NO INCREASE in 11-deoxycortisol this suggests adrenal insuff
28
what is another name for adrenogenital syndrome?
congenital adrenal hyperplasia
29
what is congenital adrenal hyperplasia?
autosomal recessive disorder of adrenal biosynthetic enzymes
30
which enzyme is most often deficient in congenital adrenal hyperplasia?
21-hydroxylase (90-95% of cases)
31
what are the 3 different types of enzyme deficiencies in congenital adrenal hyperplasia?
1. 21-hydroxylase def (most common) 2. 11-hydroxylase 3. 17-hydroxylase def
32
what is 17-hydroxylase def assoc w/?
hypogonadism
33
what happens to ACTH in congenital adrenal hyperplasia?
increases
34
what happens to the enzyme precursors in congenital adrenal hyperplasia?
the precursors proximal to the enzyme block will accumulate increased 17-ketosteroids testosterone
35
why do newborn girls with congenital adrenal hyperplasia have ambiguous genitalia?
because of increased androgen production
36
if a baby girl comes out with ambiguous genitalia, what do you do?
CHROMOSOMAL ANALYSIS
37
how is puberty affected in congenital adrenal hyperplasia?
precocious puberty (in M & F)
38
what is the growth pattern of children with congenital adrenal hyperplasia?
rapid growth as children
39
what do adults w/ congenital adrenal hyperplasia look like?
short stature
40
which 2 deficiencies of congenital adrenal hyperplasia result in ambiguous genitalia &/or weird growth?
21 & 11 hydroxylases
41
which hydroxylase deficiency of congenital adrenal hyperplasia results in DECREASED glucocorticoids & androgens?
17-hydroxylase deficiency
42
what happens to males & females with 17-hydroxylase deficiency?
F: delayed menarche & secondary sexual characteristics M: pseudohermaphroditism
43
what 3 physical findings are involved in a workup for ambiguous genitalia?
1. clitoris hypertrophy 2. poor development of labial structure 3. no palpable gonad
44
what are the 2 types of adrenocortical hyperfxn?
1. Cushing's syndrome | 2. Hyperaldosteronism
45
what is the diff btwn cushing's syndrome & dz?
``` SYNDROME = high cortisol & the assoc sx DZ = high ACTH, which RESULTS in high cortisol ```
46
what are 4 possible causes of Cushing's syndrome?
1. corticosteroid use 2. cushing's dz 3. hyperfxnal adrenal adenoma 4. ectopic ACTH production
47
what is Conn's syndrome?
hyperaldosteronism d/t hyperfxnal adrenal adenoma
48
what are the 6 sx assoc w/ Cushing's syndrome?
1. hirsutism 2. rounded face 3. hump 4. easy bruising 5. abdominal weight gain 6. abdominal striae
49
why do Cushing's pts gain weight?
hyperinsulinism secondary to hyperglycemia
50
where does fat deposit in Cushing's pts?
face nape (hump) trunk
51
why do Cushing's pts get hirsutism?
increased androgens
52
why do Cushing's pts get abdominal striae?
weakened collagen + BV rupture
53
why do Cushing's pts have diastolic HTN?
increased mineralocorticoids
54
why do Cushing's pts have muscle weakness?
muscle breakdown for gluconeogenesis
55
what will you find on a urine test of a Cushing's pt?
increased urine free cortisol
56
what happens if you give a Cushing pt a dexamethasone suppression test?
low-dose cannot suppress cortisol | high-dose can suppress in cushing DZ ONLY (not syndromes!)
57
what happens to pH in Cushing pts?
hypokalemic metabolic alkalosis
58
what happens to Cushing pts' blood sugar?
hyperglycemia
59
what zone might an ALDOSTERONE-secreting adrenal adenoma be found in?
zona glomerulosa
60
what are the 2 clinical findings in Conn's syndrome?
diastolic HTN | muscle weakness
61
what are the 4 lab findings in Conn's syndrome?
hypernatremia hypokalemia alkalosis decreased plasma renin
62
what are the 2 types of adrenal MEDULLARY hyperfxn?
1. pheochromocytoma | 2. neuroblastoma
63
what is a pheochromocytoma?
catecholamine-secreting tumor of the adrenal medulla (epi & NE)
64
what is the rule of 10s for pheo?
10% bilateral 10% malignant 10% extramedullary
65
what is a paraganglioma?
a manifestation of pheo which only produces NE | no phenylethamin-N-methyltransferase
66
what is pheo assoc w/?
NF-1 MEN IIa & IIb von hippel lindau
67
what are the physical findings of pheo related to?
increased sympathetic tone
68
what are the 7 physical findings of pheo?
1. paroxysmal &/or sustained systolic HTN w/ palpitations 2. HA 3. sweating 4. anxiety 5. chest pain 6. orthostatic intolerance 7. constipation
69
what is the best CONFIRMATORY test for pheo?
plasma free metanephrines
70
what is the best SENSITIVITY test for pheo?
24 hour urine metanephrines
71
how do pheo pts react to clonidine?
no suppression
72
what is the blood sugar of pheo pts like?
hyperglycemia
73
how can you stabilize a pheo pt pre-operatively?
alpha inhibitor & beta blocker | you HAVE to do this
74
what is neuroblastoma?
malignant neoplasm of post-ganglionic sympathetic neurons
75
what age group is commonly affected by neuroblastoma?
<5 yo
76
what is the marker of aggressive behavior in neuroblastoma?
N-MYC amplification
77
what % of neuroblastoma cases have N-MYC amplification?
20-30%
78
what % of neuroblastomas are metastatic at dx?
70% :(
79
what group has a GOOD prognosis in neuroblastoma?
<1 yo | hyperdiploid
80
what group has a BAD prognosis in neuroblastoma?
>1 yo | 1p deletion
81
how does neuroblastoma look on histo?
small round blue cell tumor
82
how does neuroblastoma look on physical exam?
large, palpable abdominal mass
83
what happens to the urine of neuroblastoma pts?
increased urine VMA & HVA
84
what is the overall survival w/ neuroblastoma & what does it depend on?
40% | depends heavily on prognostic factors

Block 10 Week 2 (6 decks)

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