Adrenal disorders

Question 1

2 main causes of primary adrenal insufficiency

Answer 1

addisons disease

adrenal enzyme defects eg congenital adrenal hyperplasia - 21 hydroxylase deficiency

Question 2

6 “I” causes of addisons disease

Answer 2

Immune destruction 
iatrogenic 
infection 
invasion 
infarction 
infiltration

Question 3

What antibodies are found in Addison’s?

Answer 3

adrenal autoantibodies - to 21-OHase

Question 4

common symptoms of primary adrenal failure

Answer 4

tiredness, weight loss, anorexia, fatigue, weakness
vomiting and diarrhoea 
skin pigmentation or vitiligo 
hypotension 
salt craving 
postural symptoms

Question 5

4 possible clues to diagnose adrenal failure

Answer 5

unexplained hypoglycaemia
disproportion between severity of illness and circulatory collapse
other endocrine features eg body hair loss, amenorrhoea
previous depression or weight loss

Question 6

Diagnosis of adrenal insufficiency - addisons

Answer 6

non specific symptoms
U+E, glucose, FBC
random cortisol
synacthen test

Question 7

If the patient is unwell and addisons suspicion is high what do you do?

Answer 7

treat with steroids

do synacthen test later

Question 8

What does the synacthen test help determine?

Answer 8

primary or secondary adrenal insufficiency

Question 9

If plasma ACTH is elevated in rapid ACTH test is this primary or secondary adrenal insufficiency?

Answer 9

primary

Question 10

If plasma ACTH is suppressed in rapid ACTH test is this primary or secondary adrenal insufficiency?

Answer 10

secondary

Question 11

Glucocorticoid replacements for adrenal insufficiency

Answer 11

Hydrocortisone
prednisolone
dexamethasone

Question 12

Mineralocorticoid replacement

Answer 12

synthetic steroid - fludrocortisone

Question 13

How does fludrocortisone work?

Answer 13

bind to aldosterone receptors

Question 14

How are mineralocorticoid doses adjusted?

Answer 14

according to U+E, clinical status eg bp, oedema and plasma renin level

Question 15

Who needs special care in terms of stress and steroids?

Answer 15

hypoadrenal patients on replacement steroids

steroid doses sufficient to suppress pituitary adrenal axis

Question 16

What action should be taken with steroids in
a - short lived illness
b - major illness or operation

Answer 16

a - double glucocorticoid dose
b - 11mh HC iv stat or 50-100mg HC iv 8 hourly and reduce HC 50% per day as symptoms resolve until previous dose is reached

Question 17

3 important self care rules for people on steroids

Answer 17

do not miss a dose
double HC dose in intercurrent illness
severe vomit or diarrhoea = call for help for intramuscular HC

Question 18

primary hyperaldosteronism causes of hypertension

Answer 18

unilateral adenoma

bilateral hyperplasia

Question 19

Rarer endocrine causes of hypertension

Answer 19

phaechromocytoma 
cushings 
acromegaly 
hyperparathyroidism 
hypothyroidism 
congenital adrenal hyperplasia

Question 20

Cortex - hypersecretion syndromes

Answer 20

cushings - cortisol and androgens

conn’s - aldosterone

Question 21

Causes of cushings syndrome

Answer 21

adenoma, carcinoma or bilateral hyperplasia

Question 22

Causes of conns syndrome

Answer 22

adenoma, bilateral hyperplasia

Question 23

medulla - hypersecretion syndromes

Answer 23

phaechromocytoma

Question 24

Symptoms of cushings syndrome

Answer 24

central obesity 
easily bruised 
hypertension 
moon face 
hirsutism 
purple striae 
oedema 
glucose intolerance 
amenorrhoea etc

Question 25

2 causes of ACTH dependent cushings

Answer 25

pituitary tumour = cushings disease

ectopic ACTH secretion eg lung carcinoid

Question 26

2 causes of ACTH independent cushings

Answer 26

adrenal tumour - adenoma, carcinoma

CCS therapy eg IBD, asthma

Question 27

Screening test for cushings

Answer 27

overnight dex test

24 hour urine free cortisol

Question 28

Confirmation test for cushings

Answer 28

24 hour urine free cortisol

low dose dex test

Question 29

Localising tumour in cushings

Answer 29

MRI sella, CT adrenal, CT chest, BIPSS

Question 30

What ratio is used in conns syndrome?

Answer 30

plasma aldosterone

plasma renin

Question 31

If PA/PRA >20 is there primary or secondary hyperaldosteronism?

Answer 31

primary

Question 32

If PA/PRA <20 is there primary or secondary hyperaldosteronism?

Answer 32

secondary

essential hypertension

Question 33

Causes of secondary hyperaldosteronism with increased PRA, PCA

Answer 33

diuretic, renin secreting tumour, coarctation aorta

Question 34

Causes of PRA and PCA reduction

Answer 34

congenital adrenal hyperplasia

exogenous mineralocorticoid

Question 35

Screening test for hyperaldosteronism

Answer 35

PRA/PCA ratio

Question 36

24 hour test for hyperaldosteronism

Answer 36

24 hour urine aldosterone and sodium during 4 days of salt loading

Question 37

Scans for hyperaldosteronism

Answer 37

CT scan adrenals
upright postue test
plasma 18-hydroxycorticosterone

Question 38

Phaechromocytoma symptoms/signs

Answer 38

hypertension

paroxysmal attacks - sweating, tremor, pallor, anxiety, headache

Question 39

Aetiology of phaechromocytoma

Answer 39

10% tumour - extra adrenal, malignant, multiple, hyperglycaemia
30% inherited - GENETIC TESTING

Question 40

Phaechromocytoma investigations

Answer 40

24 hour urine catecholamines
localise adrenal on MRI/CT
PET
remove tumour

Question 41

General investigation of adrenal mass

Answer 41

free metanephrine in plasma/urine
dex test, ALD/PRA ratio, potassium
CT, MRI, surgery

Question 42

What is the main cause of congenital adrenal hyperplasia?

Answer 42

21-hydroxylase deficiency

Question 43

What occurs in severe cases of CAH?

Answer 43

neonatal salt losing crisis

ambiguous genitalia in girls

Question 44

Incomplete defects in CAH

Answer 44

pseudoprecocious puberty in boys

hirsutism