Adrenal Disease

Question 1

What are the signs and symptoms of Cushing’s disease?

Answer 1

Weight gain
Hirsutism
Psychiatric effects (depression/euphoria)
Plethora
Moon faces
Proximal myopathy
Central obesity
Hypertension
Bruising
Buffallo hump
Striae
Poor would healing

Question 2

There are several tests which can be conducted to test is cortisol is elevated. Gives examples of these tests

Answer 2

24 hour urinary free cortisol
Urine cortisol:creatinine ratio
Overnight dexamethasone suppression test
Late night salivary cortisol

Question 3

How many different tests should be conducted to assess for elevated cortisol?

Answer 3

2

Question 4

Give examples of ACTH dependent causes of Cushing’s syndrome?

Answer 4

Pituitary adenomas
Ectopic ACTH
Ectopic CRH

Question 5

Give examples of ACTH independent causes of Cushing’ syndrome?

Answer 5

Adrenal adenomas
Adrenal carcinomas
Nodular hyperplasia

Question 6

What are the clinical features of primary adrenal insufficiency?

Answer 6

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

Question 7

In primary adrenal insuffcieicny there is ‘suspicious biochemistry’. What is meant by this?

Answer 7

Sodium is low
Potassium is high
Hypoglycaemia

Question 8

What test is performed to test for primary adrenal insufficiency?

Answer 8

Short synacthen test

Question 9

Describe the short synacthen test

Answer 9

Plasma cortisol is measured before and 30 minutes after an IV ACTH infection.

Question 10

Congenital adrenal hyperplasia can be caused by a range of genetic disorders relating to defects in the steroidogenic genes. What is the most common of these?

Answer 10

CYP21 deficiency

Question 11

What are the clinical features of congenital adrenal hyperplasia in women?

Answer 11

Ambiguous genitalia

Question 12

What are the clinical features of congenital adrenal hyperplasia in men?

Answer 12

Early virilisation
Adrenal crisis (hypotension, hyponatraemia)

Question 13

How is congenital adrenal hyperplasia treated?

Answer 13

Mineralocorticoid and glucocorticoid replacement

Question 14

What is late onset congenital adrenal hyperplasia?

Answer 14

Partial 21 alpha hydroxyls deficiency where. cortisol is maintained in normal range

Question 15

How is late onset congenital adrenal hyperplasia diagnosed?

Answer 15

Short synacthen test with 17OHP

Question 16

What are the clinical features of late onset congenital adrenal hyperplasia?

Answer 16

Oligomenorrhoea
Hirsutism
Reduced fertility

Question 17

In what cases is secondary hypertension more likely than essential hypertension?

Answer 17

Patient is young
Hypertension is resistant or severe
High clinical suspicion

Question 18

Hypertension combined with hypokalaemia is most likely to be caused by…?

Answer 18

Primary hyperaldosteronism

Question 19

What is the most common cause of secondary hypertension?

Answer 19

Primary hyperaldosteronism

Question 20

What is the best screening tool. for primary hyperaldosteronism?

Answer 20

Aldosterone-renin ratio

Question 21

Describe the saline suppression test

Answer 21

The patient is given 2L of saline over 4 hours and an aldosterone fo >270 pol/l at 4 hours is highly suspicious of primary hyperaldosteronism

Question 22

How can primary hyperaldosteronism be managed?

Answer 22

Surgically ( if caused by adrenal adenoma) to cure hypokalaemia and possibly also cure hypertension
Medical management with MR antagonists e.g. spironolactone or elperenone
Stop Beta blockers

Question 23

What is a pheochromocytoma?

Answer 23

A tumour of the adrenal medulla which can be malignant or benign

Question 24

What are the signs and symptoms of a pheochromocytoma?

Answer 24

Hypertension (often intermittent)
Episodes of headache, palpitations, pallor and sweating
Tremor
Anxiety
Nausea
Vomiting
Chest/abdominal pain
Crisis lasting ~15 mins and often well between crises

Question 25

Up to a quarter of phaeochromocytomas are associated with genetic conditions which predispose to tumours. Give examples of these genetic conditions

Answer 25

Neurofibromatosis type 1
MEN type 1
VHL
SGHB

Question 26

The majority of phaeochromocytomas are malignant. T/F?

Answer 26

False - the majority are being but 15-20% of these tumours are malignant

Question 27

How are phaeochromocytomas treated?

Answer 27

Alpha blockade with phenoxybenzamine or doxazosin
Beta blocker if patient is tachycardic
Surgical removal