adrenal disorders

Question 1

what is the core problem associated with cushing syndrome ?

Answer 1

excess cortisol

Question 2

what is the most common cause of cushing ?w

Answer 2

corticosteroid medication

Question 3

what is the difference between cushing disease and cushing syndrome ?

Answer 3

cushing disease is a pituitary disease

Question 4

what is the cause of menstrual irregularities in cushing syndrome ?

Answer 4

cortisol alters the GnRH release
which in turn decreases FSH and LH

Question 5

what are the broad causes of cushing syndrome ?

Answer 5

ACTH independent - glucocorticoid therapy , adrenal adenoma
ACTH dependent - cushing’s disease, ectopic ACTH secreting tumor

Question 6

how is a diagnosis of cushing’s syndrome made ?

Answer 6

24 hour urine free cortisol levels
salivary cortisol
low dose dexamethasone suppression test

Question 7

what is dexamethasone ?

Answer 7

a steroid

Question 8

how is a dexamethasone suppression test done ?

Answer 8

1- a dose of dexamethasone is given before bed - in order to suppress ACTH release
2- morning blood test done
3- normally the cortisol levels should be low , but in cushing the cortisol levels will be high

Question 9

after establish cushing syndrome what is the next best step to take ?

Answer 9

measure ACTH levels

Question 10

what is the high dose dexamethasone test ?

Answer 10

differentiates the cause of the high ACTH cushing syndrome

if the cause is due to pituitary adenoma - there will be a decrease in cortisol
if the cause is an ACTH secreting tumor - the cortisol will not decline

Question 11

what is the treatment for cushings sundrome ?

Answer 11

remove the source
use ketaconazole

Question 12

what is the function of ketoconazole ?

Answer 12

inhibits desmolase , the first step needed for the formation of cortisol

Question 13

what are the key side effects associated with ketoconazole ?

Answer 13

gynecomastia

Question 14

what is adrenal insufficiency and what are the two types ?

Answer 14

when there isn’t enough cortisol is made
1- primary adrenal insufficiency - Addison’s disease , cortisol and aldosterone are low , ACTH is high

2- secondary adrenal insufficiency - failure of pituitary release of ACTH - no stimulus for the release of cortisol , only cortisol is low

Question 15

what are the symptoms of adrenal insufficiency ?

Answer 15

postural hypotension
hypoglycemia
hyperkalemia
acidosis
hypovolemia

Question 16

what is the classic scenario associated with Addison’s hyper pigmentation ?

Answer 16

GI symptoms
darkening of the skin

Question 17

what is adrenal crisis ?

Answer 17

abrupt loss of cortisol and aldosterone
main manifestation is shock

Question 18

when does an adrenal crisis occur ?

Answer 18

patients who are already on long term glucocorticoid therapy and then undergo stress - surgery or infection
a sudden need for cortisol is needed

Question 19

how is adrenal crisis prevented?

Answer 19

stress dose steroids

Question 20

what are the cause of Addison disease ?

Answer 20

autoimmune - antibodies to the 21 hydroxylase enzyme
infections - TB , fungal CMV

Question 21

what is the waterhouse friderichsen syndrome and what is the cause?

Answer 21

rare cause of acute adrenal insufficiency
caused by hemorrhage in the adrenal glands

Question 22

what is waterhouse - friderichsen usually associated with ?

Answer 22

meningococcemia
non blanching rash

Question 23

what is the classic case associated with waterhouse friderichsen ?

Answer 23

patient with bacterial meningitis
acute onset of shock

Question 24

what is the most common cause of secondary adrenal insufficiency ?

Answer 24

glucocorticoid therapy due to chronic suppression of ACTH release
leads to adrenal atrophy
sudden discontinuation - hypoadrenalism

Question 25

what can help differentiate 1ry from 2ry adrenal insufficiency clinically ?

Answer 25

there is no skin hyper-pigmentation in 2ry (ACTH is low) no hyperkalemia - aldosterone is not affected

Question 26

what are the diagnostic tests for adrenal insufficiency ?

Answer 26

8 AM serum cortisol - levels should be high Serum ACTH - low levels suggest 2ry adrenal insufficiency ACTH stimulation test

Question 27

what are the different results of ACTH stimulation test and what do they indicate ?

Answer 27

exogenous ACTH is given failure to rise - primary adrenal insufficiency normal rise - secondary disorder

Question 28

what are the most common causes of primary aldosteronism ?

Answer 28

bilateral idiopathic aldosteronism aldosterone producing adenoma ( conns syndrome)

Question 29

how is a diagnosis of primary aldosteronism made ?

Answer 29

plasma renin activity plasma aldosterone conc

Question 30

how are the results from measuring the plasma renin activity evaluated ?

Answer 30

low PRA and high PAC - 1ry aldosteronism high PRA and high PAC - 2ry aldosteronism

Question 31

what are the common causes of 2ry aldosteronism ?

Answer 31

renal artery stenosis CHF Low volume

Question 32

what are the treatment options for 1ry aldosteronism ?

Answer 32

surgical adrenalectomy spironolactone

Question 33

what are phaeochromocytomas ?

Answer 33

catecholamine secreting tumors affection of chromaffin cells of the adrenal medulla

Question 34

what is the clinical presentation of pheochromocytoma ?

Answer 34

classically episodic symptoms paroxysmal headaches hypertension headaches

Question 35

how is a diagnosis of pohechromocytoma made ?

Answer 35

24 hour urine collection of metanephrines

Question 36

what is the definitive treatment for pheochromocytoma ?

Answer 36

surgery phenoxybenzamine ( alpha blockade) non selective beta blocker alpha blockade must come first

Question 37

what is a neuroblastoma ?

Answer 37

derived from neural crest cells tumor of sympathetic ganglion cells

Question 38

what gene mutation is associated with neuroblastoma and what age group is associated ?

Answer 38

n-myc gene almost always in children

Question 39

what is the presentation of neuroblastoma ?

Answer 39

usually present with abdominal pain in children opsoclonus myoclonus ataxia - dancing eyes violaceous eyelids

Question 40

how is a diagnosis of neuroblastoma made ?

Answer 40

urinary HVA VMA levels

Question 41

what is the prognosis of neuroblastoma ?

Answer 41

the younger the patient the better children over 18 usually die amplification of n myc - poor prognosis

Question 42

what imaging is used for the diagnosisi of phaeochromocytoma and neuroblastoma ?

Answer 42

MIBG

Question 43

what precautions must be taken in MIGB scan ?

Answer 43

thyroid must be protected give potassium iodidie

Question 44

what are the most important features for cushingoid appearance ?

Answer 44

purple striae on the abdomen osteoporosis ( compression fracture of the vertebrae) avascular necrosis of the femoral head

Question 45

what is the presentation of cushing in children ?

Answer 45

precocious puberty

Question 46

what is the classic case associated with adrenal crisis and what is the appropriate treatment ?

Answer 46

patient who has an autoimmune disease that is being treated with prednisolone goes into surgery - high BP drop give IV hydrocortisone

Question 47

what is the presentation of familial hyperaldosteronism ?

Answer 47

increased aldosterone levels increased sodium and water retention no mention of renin family history