Adrenal

Question 1

Classically, CAH results from a mutation in which enzyme?

Answer 1

CYP21A2

Question 2

CAH presents how in infants?

Answer 2

Virilisation of females and precocious puberty in males

Question 3

Classical symptoms for Cushing’s Disease include (8)

Answer 3

1) Acne
2) Cushingoid face
3) Hirsutism
4) Abdominal striae
5) Osteoporosis
6) Hypertension
7) Impaired glucose tolerance
8) Erectile Dysfunction

Question 4

The most common cause of Cushing’s Disease is….

Answer 4

Tumour of the corticotroph cells in anterior pituitary (70% of Cushing’s)

Question 5

The vast majority of tumours in Cushing’s are micro/ macro-adenomas?

Answer 5

Micro-adenomas

Question 6

Causes of ACTH-independent Cushing’s Syndrome include (2)

Answer 6

1) Adrenal adenoma / carcinoma

2) Bilateral macronodular adrenal hyperplasia

Question 7

What’s the formal diagnostic test for Cushing’s?

Answer 7

Low-dose dexamethasone suppression test

Question 8

If ACTH is low in a dexamethasone suppression test, what is the likely cause of the Cushing’s Syndrome?

Answer 8

Likely adrenal origin (as the body has lowered ACTH due to excess dexamethasone which resembles cortisol)

Question 9

If an ACTH is raised in a dexamethasone suppression test, what origins need to be considered?

Answer 9

Pituitary (CD) & ectopic ACTH production - the body has not responded to the test at all

Question 10

Which test can be used to differentiate ectopic from pituitary Cushing’s? How does it work?

Answer 10

High dose - it should suppress the pituitary somewhat but will have no effect on the ectopic production

Question 11

A rise in cortisol & ACTH on a CRH test indicates which source for Cushing’s?

Answer 11

Pituitary (an ectopic source wouldn’t be stimulated by CRH test as it isn’t on this axis)

Question 12

Cushing’s Disease leads to excessive androgens, true or false?

Answer 12

True

Question 13

How does Cushing’s lead to hypertension?

Answer 13

There are excessive mineralocorticoids (e.g. aldosterone)

Question 14

What’s the 1st line treatment in Cushing’s Disease?

Answer 14

Hypophysectomy and radiotherapy if recurrence

Question 15

Medical treatments for Cushing’s Disease include (2)

Answer 15

1) Ketoconazole (hepatotoxic)

2) Somatostatin analogues

Question 16

If Cushing’s is pituitary in origin, a low dose test will have what effect on ACTH?

Answer 16

None recognisable

Question 17

In a high-dose dexamethasone test, what effect will there be on ACTH production in pituitary Cushing’s?

Answer 17

ACTH will be reduced

Question 18

If ACTH production is not altered by high or low dose suppression testing, what is the likely source?

Answer 18

Ectopic

Question 19

What effect does high-dose dexamethasone have on adrenal-origin ACTH?

Answer 19

None (c.f. pituitary which is suppressed)

Question 20

Chronic Adrenal Insufficiency is also known as

Answer 20

Addison’s Disease

Question 21

Acute-hypofunction of the adrenal gland due to ischaemia is termed

Answer 21

Waterhouse-Friderichsen Syndrome

Question 22

Adenocortical carcinoma is rare and likely to be non-functional, T/F?

Answer 22

False - they are rare but are usually functional

Question 23

Primary Hyperaldosteronism is termed

Answer 23

Conn’s Syndrome

Question 24

Spironolactone bodies is a codeword for which condition?

Answer 24

Primary hyperaldosteronism

Question 25

Pigmentation changes is characteristic of which adrenal condition?

Answer 25

Addison's Disease (ACTH contains MSH code)

Question 26

Addison's Disease tends to only present when which % of gland is destroyed?

Answer 26

>90%

Question 27

The neuroendocrine chromaffin cells secrete what?

Answer 27

Catecholamines (such as adrenaline)

Question 28

Phaeochromocytoma is a tumour which occurs where in the adrenal gland & secretes what?

Answer 28

Adrenal medulla & secretes catecholamines

Question 29

The 3 most common causes of Addison's (3)

Answer 29

1) Autoimmune adrenalitis 2) Infections 3) Metastatic malignancy

Question 30

Phaeochromocytoma is often referred to as the 10% tumour - what does this mean? (5)

Answer 30

1) 10% are extra-adrenal 2) 10% are bilateral 3) 10% are malignant 4) 10% are NOT associated with hypertension 5) 25% familial (because why not)

Question 31

Phaeochromocytoma is especially associated with which genetic condition?

Answer 31

MEN2A (RET oncogene mutation)

Question 32

The 3 distinct zones of the adrenal gland are:

Answer 32

1) Zona glomerulosa 2) Zona fasciculata 3) Zona reticularis

Question 33

Name the areas of the adrenal gland & what they secrete (6)

Answer 33

1) Zona glomerulosa -> mineralocorticoids (e.g. aldosterone) 2) Zona fasciculata -> Glucorticoids (e.g. cortisol) 3) Zona reticularis -> Sex steroids + glucorticoids. 4) Medulla = adrenaline "Go, Find Rex, Make Good Sex, it's Magic"

Question 34

Aldosterone is regulated by which system?

Answer 34

Renin-Angiotensin (in response to low BP)