adrenal gland disorders

Question 1

where are the adrenal glands located?

Answer 1

above the kidneys

Question 2

where are the hormones that regulate cortisol and androgen production produced?

Answer 2

hypothalamus and anterior pituitary

Question 3

what regulates aldosterone?

Answer 3

renin-angiotensin system and plasma potassium

Question 4

when is the Renin angiotensin system activated?

Answer 4

in response to low BP

Question 5

what does renin-angiotensin system lead to?

Answer 5

production of angiotensin II

Question 6

what is the action of cortisol upon the CNS?

Answer 6

Mood lability
Euphoria/psychosis
reduced libido

Question 7

what is the affect of cortisol on the immune system

Answer 7

decreased capillary dilatation/permeability
decreased leucocyte migration
decreased macrophage activity
decreased inflammatory cytokine production

Question 8

what is the action of cortisol on bone/connective tissue?

Answer 8

Accelerates osteoporosis
reduced Serum calcium
reduced collagen formation
reduced wound healing

Question 9

what is the affect of cortisol on the metabolic system?

Answer 9

increase blood sugar
increase lipolysis
increase proteolysis

Question 10

what is the affect of cortisol on the circulatory/renal system?

Answer 10

increased Cardiac output
increased BP
increased renal blood flow and GFR

Question 11

what is the main principles of use of corticosteroids?

Answer 11

suppress inflammation
suppress immune system
replacement treatment

Question 12

when are corticosteroids used?

Answer 12

allergic disease
inflammatory disease
malignant disease

Question 13

what does addisons disease indicate?

Answer 13

cortisol/adrenal insufficiency

Question 14

what conditions are caused by primary adrenal insufficiency?

Answer 14

addisons disease
congenital adrenal hyperplasia
adrenal TB/malignancy

Question 15

what are the classical features of addisons disease?

Answer 15

Anorexia, weight loss
fatigue/lethargy
Dizziness and low BP
Abdominal pain, vomiting,
diarrhoea
Skin pigmentation

Question 16

in what % of cases of addisons are autoantibodies involved?

Answer 16

70%

Question 17

is addisons associated with other autoimmune diseases?

Answer 17

yes

Question 18

how is addisons diagnosed?

Answer 18

low sodium
high postassium
short synthetic ACTH test
increased renin and low aldosterone
adrenal antibodies

Question 19

how is adrenal insufficiency managed?

Answer 19

hydrocortisone as cortisol replacement (if unwell give via IV first)
fludrocortisone as aldosterone replacement
education

Question 20

what is the treatment in adrenal crisis?

Answer 20

fluids (saline)
steroids IV/IM hydrocortisone
(50mg/6hrs)

IF IN DOUBT TREAT

Question 21

what causes secondary adrenal insufficiency?

Answer 21

pituitary/hypothalamic disease tumours
exogenous steroid use (most common cause)

Question 22

what are the clinical features of secondary adrenal insufficiency?

Answer 22

similar to addisons EXCEPT
-pale skin (no raised ACTH)
-aldosterone production intact

Question 23

how is secondary adrenal insufficiency treated?

Answer 23

hydrocortisone
No fludrocortisone necessary

Question 24

what is caused by excess cortisol secretion?

Answer 24

cushings disease

Question 25

what are the clinical features of cushings?

Answer 25

easy bruising thin skin striae proximal myopathy

Question 26

how do we screen for cortisol excess?

Answer 26

Overnight dexamethasone suppression test 24 hour urinary free cortisol Late night salivary cortisol Repeat to confirm

Question 27

what is the diagnostic test for cushings syndrome?

Answer 27

low dose dexomethasone suppression test

Question 28

what are the implications of atrophy of the adrenal cortex?

Answer 28

Unable to respond to stress (illness/surgery) Need extra doses of steroid when ill/surgical procedure Cannot stop suddenly Gradual withdrawal of steroid therapy if >4-6 week

Question 29

what can indicate primary aldosterone?

Answer 29

hypertension and hypokalaemia (hypokalaemia is only in around 30% of cases though)

Question 30

what is the definition of primary aldosterone?

Answer 30

autonomous over production of aldosterone independent of its regulators

Question 31

does aldosterone increase BP?

Answer 31

yes

Question 32

what is the most common type of primary aldosterone?

Answer 32

bilateral adrenal hyperplasia (60% of cases)

Question 33

how is aldosterone excess confirmed?

Answer 33

Measure plasma aldosterone and renin and express as ratio (ARR-aldosterone to renin ratio) → If ratio raised (assay dependent) then investigate further with saline suppression test → Failure of plasma aldosterone to suppress by > 50% with 2 litres of normal saline confirms PA

Question 34

how do we confirm which subtype of primary aldosterone a patient has?

Answer 34

Adrenal CT to demonstrate adenoma → Sometimes adrenal vein sampling to confirm adenoma is true source of aldosterone excess

Question 35

how is primary aldosterone managed surgically?

Answer 35

Unilateral laparoscopic adrenalectomy → Only if adrenal adenoma (and excess confirmed in adrenal vein sampling) → Cure of hypokalaemia → Cures hypertension in 30-70% cases generally only used in those with a clear adenoma

Question 36

how is primary aldosterone managed medically?

Answer 36

generally only used in bilateral adrenal hyperplasia Use MR antagonists (spironolactone or eplerenone)

Question 37

what is congenital adrenal hyperplasia?

Answer 37

an inherited group of disorders characterised by a deficiency in one of the enzymes necessary for cortisol synthesis

Question 38

when is classical CAH diagnosed?

Answer 38

in infancy

Question 39

when is non classical CAH usually diagnosed?

Answer 39

in adolescence/adulthood

Question 40

what are the symptoms of non classical CAH?

Answer 40

hirsutism, menstrual disturbance and infertility due to anovulation

Question 41

what are the symptoms of classical CAH?

Answer 41

virilisation/salt wasting

Question 42

how is CAH diagnosed?

Answer 42

Basal [or stimulated] 17-OH Progesterone increasingly supported by genetic mutation analysis

Question 43

what is the presentation of classical CAH?

Answer 43

Adrenal insufficiency → Often around two to three weeks → Poor weight gain → Biochemical pattern of Addison’s disease Females → Genital ambiguity (virilisation)

Question 44

what is the presentation of non classical CAH (typically in females)

Answer 44

Hirsute Acne Oligomenorrhoea Precocious puberty Infertility or sub-fertility

Question 45

what is the main treatments of CAH in childhoof?

Answer 45

Timely recognition Glucorticoid replacement Mineralocorticoid replacement in some Surgical correction Achieve maximal growth potential

Question 46

what is the main treatments of CAH in adults?

Answer 46

Control androgen excess Restore fertility Avoid steroid over-replacement

Question 47

what are things that indicate phaeochromocytoma?

Answer 47

Labile hypertension * Postural hypotension * Paroxysmal sweating, headache, pallor, tachycardia Also none of the above!

Question 48

why is phaeochromocytoma known as the 10% tumour?

Answer 48

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

Question 49

when are 50% of phaeochromocytomas diagnosed?

Answer 49

during post mortem

Question 50

what is the classic triad of symptoms in phaeochromocytoma?

Answer 50

hypertension headache sweating up to 90% of cases

Question 51

what are the signs of phaeochromocytoma?

Answer 51

hypertension pallor brady/tachycardia pyrexia

Question 52

how is phaeochromocytoma diagnosed?

Answer 52

through identifying the source of catecholamines MRI scan MIBG- meta iodobenzylguanidine

Question 53

how would a phaeochromocytoma be treated?

Answer 53

SURGICAL MANAGEMENT preparation for surgery- Full α and β- blockade (A before B) Phenoxybenzamine (α-blocker) Propranolol, atenolol or metoprolol (β- blocker)