Adrenal Disease

Question 1

what are the two main adrenal medullary tumours?

Answer 1

neuroblastoma

pheochromocytoma

Question 2

from what cells is pheochromocytoma derived?

Answer 2

chromaffin cells of the adrenal medulla

Question 3

what does pheochromocytoma secrete?

Answer 3

catecholamines

Question 4

why is pheochromocytoma called the 10% tumour?

Answer 4

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

Question 5

which adrenal disorder is associated with dark skin?

Answer 5

addison’s disease (high ACTH)

Question 6

what are the main primary adrenal insufficiencies?

Answer 6

addison’s disease
congenital adrenal hyperplasia
malignancy
adrenal TB

Question 7

what is the most common cause of primary adrenal insufficiency?

Answer 7

addison’s disease

Question 8

is addison’s disease an autoimmune disease?

Answer 8

yes

associated with autoantibodies in the majority of cases

Question 9

list clinical features of addison’s disease

Answer 9

dizziness
low BP 
fatigue 
anorexia 
skin pigmentation

Question 10

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

Answer 10

low sodium

high potassium

Question 11

which diagnostic test is used for Addison’s disease?

Answer 11

short synACTHen test

Question 12

outline management for adrenal insufficiency

Answer 12

hydrocortisone 30mg

fludrocortisone

Question 13

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

Answer 13

false

never stop suddenly, but the dose can be reduced

Question 14

list causes of secondary adrenal insufficiency

Answer 14

pituitary/hypothalamic tumours

exogenous steroid use

Question 15

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

Answer 15

suppress CRH and ACTH

Question 16

what hormone is in excess in cushing’s syndrome?

Answer 16

cortisole

Question 17

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

Answer 17

false

Question 18

list clinical features of cushing’s syndrome

Answer 18

moon face 
abdominal striae 
thin skin 
easy bruising 
proximal myopathy 
osteoporosis

Question 19

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

Answer 19

pituitary disease/adenoma

ectopic tumours

Question 20

what are ACTH independent causes of cushing’s syndrome?

Answer 20

adrenal disease/adenoma

nodular hyperplasia

Question 21

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

Answer 21

low dose dexamethasone suppression test

Question 22

what is the most common cause of cortisol excess?

Answer 22

high dose steroid therapy

Question 23

what is primary aldosteronism?

Answer 23

autonomous production of aldosterone independent of its regulators

Question 24

which syndrome occurs due to aldosteronism?

Answer 24

conn’s syndrome

Question 25

what is the most common cause of secondary hypertension?

Answer 25

primary aldosteronism

Question 26

outline how an aldosterone excess is diagnosed

Answer 26

measure aldosterone:renin ratio if raised, do saline suppression test failure of aldosterone to suppress by 50% with 2L saline confirms aldosteronism

Question 27

what are the main causes of aldosteronism?

Answer 27

adrenal hyperplasia adrenal adenoma genetic mutations

Question 28

outline management of primary aldosteronism

Answer 28

adrenalectomy if unilateral adenoma spironolactone if bilateral hyperplasia

Question 29

what is the most common cause of congenital adrenal hyperplasia?

Answer 29

21-alpha-hydroxylase deficiency

Question 30

what three main effects does 21-alpha-hydroxylase deficiency cause?

Answer 30

low cortisol low aldosterone excess androgens

Question 31

what is the classical triad of symptoms in pheochromocytoma?

Answer 31

hypertension headache sweating

Question 32

how is pheochromocytoma diagnosed?

Answer 32

MRI MIBG PET scan

Question 33

outline therapy for pheochromocytoma

Answer 33

alpha blockers beta blockers fluid replacement surgical excision

Question 34

pheochromocytoma is associated with which clinical syndromes?

Answer 34

``` MEN 2 von hippel lindau succinate dehydrogenase mutations neurofibromatosis tuberous sclerosis ```

Question 35

which gene is affected in MEN 1?

Answer 35

MEN1 gene (11q)

Question 36

which gene is affected in MEN 2?

Answer 36

RET gene (10q)

Question 37

true or false: the MEN1 gene is a tumour suppressor

Answer 37

true

Question 38

is the RET gene a tumour suppressor or a proto-oncogene?

Answer 38

proto-oncogene

Question 39

von hippel lindau syndrome is caused by a mutation in which gene?

Answer 39

VHL gene

Question 40

list the parts of the adrenal gland

Answer 40

outer capsule cortex medulla

Question 41

what are the three zones located in the adrenal cortex?

Answer 41

zona glomerulosa zona fasciculata zona reticularis

Question 42

which cells are located in the adrenal medulla?

Answer 42

chromaffin cells

Question 43

what controls hormonal release in the zona glomerulosa?

Answer 43

RAAS (angiotensin II)

Question 44

which hormone is secrete from the zonua glomerulosa?

Answer 44

aldosterone

Question 45

what controls hormonal release in the zona fasciculata?

Answer 45

ACTH

Question 46

what hormones are secreted from the zona fasiculata?

Answer 46

cortisol | corticosterone

Question 47

what controls hormonal release in the zona reticularis?

Answer 47

ACTH

Question 48

what hormones are secreted from the zona reticularis?

Answer 48

androgens

Question 49

what controls hormonal release in the adrenal medulla?

Answer 49

sympathetic nervous system

Question 50

what hormones are secreted from the adrenal medulla?

Answer 50

catecholamines

Question 51

outline the hypothalamus pituitary adrenal cortex axis

Answer 51

HYPOTHALAMUS secretes CRH which stimulates anterior PITUITARY to secrete ACTH which stimulates the ADRENAL GLAND to secrete cortisol

Question 52

which part of the H-P-adrenal cortex axis does cortisol inhibit in negative feedback?

Answer 52

hypothalamus | anterior pituitary

Question 53

which system regulates aldosterone release?

Answer 53

RAAS

Question 54

what is the effect of cortisol upon the CNS?

Answer 54

varying mood psychosis loss of libido

Question 55

what is the effect of cortisol upon bone?

Answer 55

osteoporosis

Question 56

what is the effect of cortisol upon metabolism?

Answer 56

increases breakdown of carbs, lipids and proteins

Question 57

what are the main effects of aldosterone?

Answer 57

increased K + H ion excretion | sodium retention