Adrenal Disorders (Hypo)

Question 1

What does the adrenal cortex make?

Answer 1

Corticosteroids

Question 2

What is the definition of a steroid?

Answer 2

Comes from choelstrol

Question 3

Where in the adrenal gland are corticosteroids made?

Answer 3

Adrenal cortex

Question 4

What corticosteroids are made in the adrenal cortex?

Answer 4

1. Mineralocorticoids (Aldosterone)
2. Glucocorticoids (Cortisol)
3. Sex steroids (Androgens, oestrogens)

Question 5

What adrenal enzymes does angiotensin II activate?

Answer 5

1. Side Chain Cleavage
2. 3 Hydroxysteroid dehydrogenase
3. 21 hydroxylase
4. 11 hydroxylase
5. 18 hydroxylase

Question 6

What is aldosterone’s action?

Answer 6

Controls blood pressure, sodium and lowers potassium

Question 7

What adrenal enzymes does ACTH active?

Answer 7

1. Side Chain Cleavage
2. 3 Hydroxysteroid dehydrogenase
3. 21 hydroxylase
4. 11 hydroxylase
5. 17 hydroxylase

Question 8

What enzyme has diurnal rhythm?

Answer 8

Cortisol

Question 9

What is primary adrenal failure called?

Answer 9

Addison’s disease

Question 10

What is Addison’s disease caused by in the UK?

Answer 10

Autoimmune disease where the immune system decides to destroy the adrenal cortex

Question 11

What is commonest cause of Addison’s disease worldwide?

Answer 11

Tuberculosis

Question 12

What does the pituitary secrete in Addison’s disease?

Answer 12

ACTH and hence MSH

Question 13

Why does an Addison’s patient have low blood pressure?

Answer 13

No cortisol or aldosterone

Question 14

What skin condition may coexist with Addison’s disease?

Answer 14

Autoimmune vitiligo

Question 15

Why does an Addison’s patient have increased pitgmentation?

Answer 15

POMC is a large precursor protein that is cleaved to form a number of smaller peptides, including ACTH, MSH and endorphins

Question 16

What are the pathophysiological causes of adrenocortical failure?

Answer 16

1. Adrenal glands destroyed (2)
2. Enzymes in the steroid synthetic
   pathway not working (1)

Question 17

What are the causes of adrenocortical failure?

Answer 17

1. Tuberculous Addison’s disease 
(commonest worldwide)
2. Autoimmune Addison’s disease 
(commonest in UK)
3. Congenital adrenal hyperplasia

Question 18

What are the consequences of adrenocortical failure?

Answer 18

1. Fall in blood pressure
2. Loss of salt in the urine
3. Increased plasma potassium
4. Fall in glucose due to glucocorticoid deficiency
5. High ACTH resulting in increased pigmentation
6. Eventual death due to severe hypotension

Question 19

What is POMC?

Answer 19

Pro-opio melanocortin

Question 20

Where is POMC synthesized?

Answer 20

Pituitary

Question 21

What is POMC broken down into?

Answer 21

ACTH
MSH
Endorphins
Enkephalins
Other peptides

Question 22

What tests do you preform once there is clinical suspicion for Addision’s?

Answer 22

1. 9am cortisol = low
2. ACTH = high
3. Short synACTHen test
   - Give 250 ug synacthen IM
   - Measure cortisol response

Question 23

How does an Addison’s patient respond to synACTH?

Answer 23

Cortisol at 9am = 100 (270-900)
Administer injection IM of synacthen
Cortisol at 9.30 = 150 (>600)nM

Question 24

What is the treatment of adrenal failure?

Answer 24

1. Prednisolone 3mg daily
   or
   Hydrocortisone three times daily (10 + 5 + 2.5)
2. Fludrocortisone 50 to 100 mcg daily

Question 25

What is congenital adrenal hyperplasia most commonly caused by?

Answer 25

21-hydroxylase deficiency

Question 26

Which hormones will be totally absent in complete 21-hydrosylase deficiency?

Answer 26

Aldosterone and cortisol

Question 27

How long can you survive in complete 21-hydrosylase deficiency?

Answer 27

Less than 24hrs

Question 28

Which hormones will be in excess in complete 21-hydrosylase deficiency?

Answer 28

Sex steroids and testosterone

Question 29

What is the presentation of complete 21-hydrosylase deficiency?

Answer 29

As a neonate with a salt losing Addisonian crisis

Question 30

Why is the foetus not affected by complete 21-hydrosylase deficiency in utero?

Answer 30

Foetus gets steroids across placenta

Question 31

How may infant girls be affected by complete 21-hydrosylase deficiency?

Answer 31

Might have ambiguous genitalia (virilised by adrenal testo)

Question 32

Which hormones are deficient in partial 21-hydrosylase deficiency?

Answer 32

Cortisol and aldosterone

Question 33

Which hormones will be in excess in partial 21-hydrosylase deficiency?

Answer 33

Sex steroids and testosterone

Question 34

At what age will they present?

Answer 34

Any age, as they survive

Question 35

What is the main problem in later life in partial 21-hydroxylase deficiency for girls?

Answer 35

Hirsutism and virilisation

Question 36

What is the main problem in later life in partial 21-hydroxylase deficiency for boys?

Answer 36

Precocious puberty

Question 37

What is the main problem in later life in partial 21-hydroxylase deficiency due to?

Answer 37

Adrenal testosterone