Hyperadrenal disorders

Question 1

What are the clinical features of Cushing’s syndrome?

Answer 1

• Excess cortisol
• Centripetal obesity
• Moon face + buffalo hump
• Proximal myopathy
• Hypertension + hypokalaemia
• Red striae, thin skin + easy bruising
• Osteoporosis
• Diabetes

Question 2

What are the causes of Cushing’s syndrome?

Answer 2

EXCESS CORTISOL

• Pituitary dependent Cushing’s disease
• Ectopic ACTH from lung cancer
• Adrenal adenoma making corisol
• Oral glucocorticoids

Question 3

What investigations are carried out for Cushing’s syndrome?

Answer 3

1. 24h urine collection for urinary free cortisol
2. Blood diurnal cortisol levels
   - If high at midnight –> Cushing’s
3. Low dose dexamethasone suppression test (gold standard):
   - Dexamethasone

Question 4

How is Cushing’s syndrome treated?

Answer 4

1st - medical so patient is safe for operation:

• Metyrapone (11B-hydroxylase inhibitor)
• Ketoconazole (CYP450 side chain cleavage enzyme inhibitor)
• Receptor blocking drugs

Then surgery: depends on cause:

• Transphenoidal hypophysectomy if pituitary-dependent
• Bilateral adrenalectomy
• Unilateral arednalectomy for adrenal mass

Question 5

What are the clinical features of Conn’s syndrome?

Answer 5

• Excess aldosterone
• Hypertension
• Hypokalaemia

Question 6

What is the cause of Conn’s syndrome?

Answer 6

Benign adrenocortical tumour (zone glomerulosa - makes aldosterone)

Question 7

How is Conn’s syndrome diagnosed?

Answer 7

1. High blood pressure
2. Blood test - low potassium
3. Measure aldosterone - high
4. Measure renin - low (hypertension suppressing RAS)

Question 8

How is Conn’s syndrome treated?

Answer 8

1. Mineralocorticoid receptor antagonist - spironolactone
2. Surgery to remove tumour
   - If bilateral adrenal hyperplasia, both adrenals are not removed, tf long-term eplerenone (also MR antagonist, fewer side-effects) post-surgery

Question 9

What are the clinical features of phaeochromocytoma?

Answer 9

• Episodic severe hypertension in young people

- Can lead to stroke, MI, sudden cardiac death (VF)

Question 10

How is phaeochromocytoma managed?

Answer 10

1. Alpha blocker - block effects of excess catecholamines (vasoconstriction –> hypertension)
2. Once BP is normalised, beta blocker to prevent tachycardia
3. Surgery to remove tumour

Question 11

What is phaeochomocytoma?

Answer 11

Tumour of adrenal medulla that leads to the over-production of catecholamines