Disorders Of Adrenocortical Function

Question 1

What is congenital adrenal hyperplasia?

Answer 1

Impaired cortisol synthesis due to a deficiency of one of the 5 enzymes needed for cortisol synthesis

Question 2

Where are adrenaline and noradrenaline made?

Answer 2

Adrenal medulla

Question 3

What are the three stages of cushings disease investigation?

Answer 3

Screening, confirmation and differentiation of the cause

Question 4

What are the three differential diagnosis for positive screening?

Answer 4

True Cushing’s syndrome, pseudo Cushing’s syndrome and exogenous steroids

Question 5

What are the symptoms of pseudocushings syndrome?

Answer 5

Depression, alcoholism, anorexia and obesity

Question 6

When does ectopic ACTH production happen?

Answer 6

When the neuroendocrine cells become malignant

Question 7

What happens in terms of aldosterone and cortisol in extreme situations (like bleeding to death)?

Answer 7

You dont have enough aldosterone so cortisol oversaturates the cortisol -> cortisone enzyme and then causes sodium retention

Question 8

What imaging do you do for a pituitary tumour?

Answer 8

MRI or inferior petrosa sinus sampling

Question 9

What imaging do you do for an adrenal tumour?

Answer 9

CT or MRI

Question 10

What imaging do you do for an ectopic tumour?

Answer 10

ACTH sampling or octreotide scan

Question 11

What is an octreotide scan?

Answer 11

Putting a nuclear tracer on somatostatin

Question 12

Why do patients need to take steroid replacement tablets when they have tumour removal surgery?

Answer 12

The tumour suppresses the glands ability for secretion, so it needs time to replenish

Question 13

What are some of the causes of addisons syndrome?

Answer 13

Autoimmune, TB, steroid withdrawal, metastasis, infiltration, apoplexy, infection, enzyme defect and drugs

Question 14

What are the four dynamic tests for adrenal insufficiency?

Answer 14

Short Synacthen, long synacthen test, insulin tolerance test and glucagon test

Question 15

What are the two treatments for Addison’s disease?

Answer 15

Hydrocortisone and fludrocortisone

Question 16

What is the most common deficiency in congenital adrenal hyperplasia?

Answer 16

21- hydroxylase

Question 17

Why is lots of testosterone produced in congenital adrenal hypoplasia?

Answer 17

No cortisol means no negative feedback and lots of ACTH -> lots of cholesterol -> lots of testosterone

Question 18

What does excess testosterone cause?

Answer 18

Virilisation, hirsutism, infertility

Question 19

What does 11 beta hydroxylase deficiency cause?

Answer 19

No aldosterone or cortisol but high deoxycorticosterone and testosterone

Question 20

How do you test for congenital adrenal hypoplasia?

Answer 20

Synacthen test or prednisone suppression

Question 21

What would you see in the synacthen test in a 11 beta hydroxylase deficient patient?

Answer 21

No cortisol rise and increased 17OH progesterone levels

Question 22

What should happen in a prednisone suppression test?

Answer 22

Androgens should fall into normal range

Question 23

How do you treat congenital adrenal hypoplasia?

Answer 23

Glucocorticoid therapy, surgery to virilised female genetalia and treatment of mother to prevent foetal virilisation

Question 24

Where is aldosterone produced?

Answer 24

Zone glomerulosa of the adrenal cortex

Question 25

What are the clinical signs of primary excess aldosterone syndromes?

Answer 25

High aldosterone and low renin

Question 26

What are the clinical signs of secondary excess aldosterone syndromes?

Answer 26

High aldosterone and high renin

Question 27

What are the names of some primary aldosterone excess syndromes?

Answer 27

Conn’s, bilateral adrenal hyperplasia, steroid treatable hypertension and aldosterone producing adrenal carcinoma

Question 28

What are the names of some secondary aldosterone excess conditions with hypertension?

Answer 28

Renal artery stenosis, renin secreting tumour, malignant nephrosclerosis

Question 29

What are the names of some secondary aldosterone excess conditions with a normal BP?

Answer 29

CCF, cirrhosis, nephrotic syndrome and dehydration

Question 30

What is a phaeochromocytoma?

Answer 30

Tumour of the enterochromaffin cells of the adrenal medulla

Question 31

Where do extra adrenal phaeochromocytomas occur?

Answer 31

In sympathetic ganglia or organ of zuckerkandl

Question 32

What catecholamines do phaeochromocytomas produce?

Answer 32

Adrenaline, noradrenaline and dopamine

Question 33

What is the rule of 10 with phaeochromocytomas?

Answer 33

10% bilateral, 10% malignant and 10% extra adrenal

Question 34

What are some phaeochromocytoma- associated syndromes?

Answer 34

Neurofibromatosis type 1, von hippel-Lindau disease, multiple endocrine neoplasia type 2, hereditary paraganglioma syndrome

Question 35

Why is the thyroid gland removed in multiple endocrine neoplasia type 2 patients?

Answer 35

Stop the formation of a medullary thyroid carcinoma

Question 36

What are the main symptoms of phaeochromocytoma?

Answer 36

Sweating, headache, tachycardia

Question 37

What are the pharmacological symptoms of phaeochromocytoma?

Answer 37

Eosinophilia, hyperglycaemia, hypercalcemia, raised urinary metonephrines and catecholamines

Question 38

What types of drugs are used to manage phaeochromocytomas?

Answer 38

Alpha and beta blockade