Adrenal Disease Flashcards

1
Q

what are the two main adrenal medullary tumours?

A

neuroblastoma

pheochromocytoma

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2
Q

from what cells is pheochromocytoma derived?

A

chromaffin cells of the adrenal medulla

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3
Q

what does pheochromocytoma secrete?

A

catecholamines

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4
Q

why is pheochromocytoma called the 10% tumour?

A

10% are extra adrenal
10% are bilateral
10% are malignant
10% are not associated with hypertension

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5
Q

which adrenal disorder is associated with dark skin?

A

addison’s disease (high ACTH)

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6
Q

what are the main primary adrenal insufficiencies?

A

addison’s disease
congenital adrenal hyperplasia
malignancy
adrenal TB

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7
Q

what is the most common cause of primary adrenal insufficiency?

A

addison’s disease

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8
Q

is addison’s disease an autoimmune disease?

A

yes

associated with autoantibodies in the majority of cases

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9
Q

list clinical features of addison’s disease

A
dizziness
low BP 
fatigue 
anorexia 
skin pigmentation
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10
Q

what would sodium and potassium levels be like in addison’s disease?

A

low sodium

high potassium

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11
Q

which diagnostic test is used for Addison’s disease?

A

short synACTHen test

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12
Q

outline management for adrenal insufficiency

A

hydrocortisone 30mg

fludrocortisone

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13
Q

true or false: patients on hydrocortisone for adrenal insufficiency can stop whenever they want

A

false

never stop suddenly, but the dose can be reduced

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14
Q

list causes of secondary adrenal insufficiency

A

pituitary/hypothalamic tumours

exogenous steroid use

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15
Q

what is the effect of exogenous steroid on the H-P-adrenal cortext axis?

A

suppress CRH and ACTH

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16
Q

what hormone is in excess in cushing’s syndrome?

A

cortisole

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17
Q

true or false: cushing’s syndrome is more common in men

A

false

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18
Q

list clinical features of cushing’s syndrome

A
moon face 
abdominal striae 
thin skin 
easy bruising 
proximal myopathy 
osteoporosis
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19
Q

what are the ACTH dependent causes of cushing’s syndrome?

A

pituitary disease/adenoma

ectopic tumours

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20
Q

what are ACTH independent causes of cushing’s syndrome?

A

adrenal disease/adenoma

nodular hyperplasia

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21
Q

what is the gold standard test for diagnosing cushing’s syndrome?

A

low dose dexamethasone suppression test

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22
Q

what is the most common cause of cortisol excess?

A

high dose steroid therapy

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23
Q

what is primary aldosteronism?

A

autonomous production of aldosterone independent of its regulators

24
Q

which syndrome occurs due to aldosteronism?

A

conn’s syndrome

25
what is the most common cause of secondary hypertension?
primary aldosteronism
26
outline how an aldosterone excess is diagnosed
measure aldosterone:renin ratio if raised, do saline suppression test failure of aldosterone to suppress by 50% with 2L saline confirms aldosteronism
27
what are the main causes of aldosteronism?
adrenal hyperplasia adrenal adenoma genetic mutations
28
outline management of primary aldosteronism
adrenalectomy if unilateral adenoma spironolactone if bilateral hyperplasia
29
what is the most common cause of congenital adrenal hyperplasia?
21-alpha-hydroxylase deficiency
30
what three main effects does 21-alpha-hydroxylase deficiency cause?
low cortisol low aldosterone excess androgens
31
what is the classical triad of symptoms in pheochromocytoma?
hypertension headache sweating
32
how is pheochromocytoma diagnosed?
MRI MIBG PET scan
33
outline therapy for pheochromocytoma
alpha blockers beta blockers fluid replacement surgical excision
34
pheochromocytoma is associated with which clinical syndromes?
``` MEN 2 von hippel lindau succinate dehydrogenase mutations neurofibromatosis tuberous sclerosis ```
35
which gene is affected in MEN 1?
MEN1 gene (11q)
36
which gene is affected in MEN 2?
RET gene (10q)
37
true or false: the MEN1 gene is a tumour suppressor
true
38
is the RET gene a tumour suppressor or a proto-oncogene?
proto-oncogene
39
von hippel lindau syndrome is caused by a mutation in which gene?
VHL gene
40
list the parts of the adrenal gland
outer capsule cortex medulla
41
what are the three zones located in the adrenal cortex?
zona glomerulosa zona fasciculata zona reticularis
42
which cells are located in the adrenal medulla?
chromaffin cells
43
what controls hormonal release in the zona glomerulosa?
RAAS (angiotensin II)
44
which hormone is secrete from the zonua glomerulosa?
aldosterone
45
what controls hormonal release in the zona fasciculata?
ACTH
46
what hormones are secreted from the zona fasiculata?
cortisol | corticosterone
47
what controls hormonal release in the zona reticularis?
ACTH
48
what hormones are secreted from the zona reticularis?
androgens
49
what controls hormonal release in the adrenal medulla?
sympathetic nervous system
50
what hormones are secreted from the adrenal medulla?
catecholamines
51
outline the hypothalamus pituitary adrenal cortex axis
HYPOTHALAMUS secretes CRH which stimulates anterior PITUITARY to secrete ACTH which stimulates the ADRENAL GLAND to secrete cortisol
52
which part of the H-P-adrenal cortex axis does cortisol inhibit in negative feedback?
hypothalamus | anterior pituitary
53
which system regulates aldosterone release?
RAAS
54
what is the effect of cortisol upon the CNS?
varying mood psychosis loss of libido
55
what is the effect of cortisol upon bone?
osteoporosis
56
what is the effect of cortisol upon metabolism?
increases breakdown of carbs, lipids and proteins
57
what are the main effects of aldosterone?
increased K + H ion excretion | sodium retention