Adrenal Disorders

Question 1

Clinical presentation of adrenal cortex disease

Presentation of adrenal medulla disease

Answer 1

Adrenal hormone deficiency

Question 2

Biochemical assessment of adrenal cortex

Answer 2

If there is suspected adrenal hormone deficiency

Question 3

Biochemical assessment of adrenal medulla

Answer 3

24h urine catecholamines - adrenaline, noradrenaline, dopamine, 3-Methoxy-Tyramine

24h urine metanephrines - metabolites of adrenaline and noradrenaline metadrenaline, normetadrenaline

plasma metanephrines - more sensitive than 24h urine
avoid certain foods before collection - coffee, coke, bananas, chocolate, vanilla

Adrenal insufficency -
Primary adrenal failure - destruction of adrenal cortex - Addi

Question 4

Primary adrenal failure (addisons disease)

Answer 4

Original cases seen by Dr addison had TB of the adrenal glands - in modern day destruction of adrenal glands is usually due to autoimmune disease

Clinical fea

Question 5

ACTH deficiency and steroid suppression

Answer 5

ACTH deficiency - Occurs in any cause of hypopituitarism

Similar symptoms to primary adrenal failure
No pigmentation as ACTH not raised
No hyperkalaemia as no mineralocorticoid deficiency
Hyponatraemia due to effect of cortisol on free water excretion

Steroid-induced hypoadrenalism
ACTH suppressed with long-term steroids
Abrupt withdrawal can cause hypo-adrenal crisis - as body is not producing cortisol
Important to consider in any unwell patient on steroids

Question 6

Cushing’s syndrome

Answer 6

Clinical syndrome due to glucocorticoid excess

Round pink face with round abdomen

Question 7

Primary hyperaldosteronism

Answer 7

Excess production of aldosterone from adrenal gland

Could be due to — Aldosterone-secreting adrenal adenoma - Conn

Question 8

Congenital adrenal hyperplasia (CAH)

Answer 8

Genetic - Rare but important inherited disorder

Autosomal recessive

Adrenal crisis and ambiguous genitalia

Caused by a block in adrenal cortex pathway

Presentation depends on enzyme defect

Lack of a certain enzyme can leads to: Low cortisol and/or aldosterone but High male hormone (androgens)

This is because formation of all hormones comes from cholesterol - if e.g. 21-hydroxylase (which helps produce aldosterone and cortisol by not androgens) is blocked, then all the metabolites will end up being made into testosterone - which in woman can be bad

Presentation - Hypotension Hyponatraemia Hyperkalaemia Hypoglycaemia Virilisation Amibiguous genitalia

Treatment - Treat adrenal crisis, Determine sex of baby
Long term - supplement glucocorticoids and mineralcortoicds + Corrective surgery

Question 9

Phaechromocytoma and paraganglioma

Answer 9

Phaeochromocytoma - Tumour of adrenal medulla
Paraganglioma - Extra-adrenal tumour -chromaffin tissue origin
90% of tumors found are from adrenal medulla, 10% extra-adrenal origin

Genetic inheritance common
More likely if: