Adrenal - Insufficiency

Question 1

In Addison’s disease there is deficiency of which hormones?

Answer 1

Glucocorticoids and mineralocorticoids

Question 2

80% of cases of Addison’s disease in the UK are due to what?

Answer 2

Autoimmunity

Question 3

What is the commonest cause of Addison’s disease worldwide?

Answer 3

TB

Question 4

What is the commonest form of congenital adrenal hyperplasia and how is it inherited?

Answer 4

21 alpha hydroxylase deficiency - autosomal recessive

Question 5

How does congenital adrenal hyperplasia present in boys?

Answer 5

Adrenal insufficiency and poor weight gain

Question 6

How does congenital adrenal hyperplasia present in girls?

Answer 6

Ambiguous genitalia

Question 7

How is congenital adrenal hyperplasia treated?

Answer 7

Glucocorticoid and mineralocorticoid replacement

Question 8

Describe a typical individual affected by Addison’s disease?

Answer 8

Tall, thin, tanned, tired

Question 9

What causes the hyperpigmentation that can be seen in Addison’s disease?

Answer 9

Raised ACTH

Question 10

What other autoimmune condition is commonly related to Addison’s disease?

Answer 10

Vitiligo

Question 11

Addison’s disease should be considered as a diagnosis in all individuals with which unexplained symptoms?

Answer 11

Abdominal pain or vomiting

Question 12

What are the most common causes of an Addisonian crisis?

Answer 12

Acute illness in those with pre-existing or undiagnosed Addison’s disease / sudden stopping of long-term steroid medication

Question 13

What happens to the levels of sodium and potassium in Addison’s disease?

Answer 13

Hyponatraemia and hyperkalaemia

Question 14

If an acid-base disorder is to be seen in those with Addison’s disease, what is it most likely to be?

Answer 14

Metabolic acidosis

Question 15

What happens to blood glucose levels in Addison’s disease?

Answer 15

Hypoglycaemia

Question 16

What happens to the levels of renin and aldosterone in Addison’s disease?

Answer 16

High renin, low aldosterone

Question 17

What is the definitive investigation for diagnosing Addison’s disease?

Answer 17

Short Synacthen test

Question 18

Addison’s disease can be excluded if the plasma cortisol is greater than what, 30 minutes after receiving IM synthetic ACTH?

Answer 18

550nmol/L

Question 19

Cortisol levels may be falsely elevated in who?

Answer 19

Pregnant women or those taking the COCP

Question 20

If the short Synacthen test cannot be readily accessed, what is the next best investigation to do for Addison’s disease?

Answer 20

9am cortisol and ACTH levels

Question 21

What happens to the 9am ACTH level in primary adrenal insufficiency (i.e. Addison’s disease)?

Answer 21

Raised

Question 22

What happens to the 9am cortisol level in secondary adrenal insufficiency?

Answer 22

Low

Question 23

A 9am plasma cortisol level of what is highly suggestive of Addison’s disease?

Answer 23

< 100nmol/L

Question 24

What is the treatment for Addison’s disease?

Answer 24

Hydrocortisone (15-20mg PO) and fludrocortisone (50-200mcg PO)

Question 25

How should hydrocortisone be given in the management of Addison's disease?

Answer 25

In 2-3 divided daily doses

Question 26

What should patients with Addison's disease do before undertaking any strenuous exercise?

Answer 26

Take 5-10mg hydrocortisone

Question 27

What should happen to the steroid doses in Addison's disease during an acute febrile illness?

Answer 27

Double the dose of hydrocortisone

Question 28

How should an Addisonian crisis be treated immediately?

Answer 28

100mg IV hydrocortisone and IV fluid bolus

Question 29

Patients in an Addisonian crisis may have what elecrolyte abnormality? What initial investigation should be done to check for this?

Answer 29

Hyperkalaemia - ECG

Question 30

What is the commonest cause of secondary adrenal insufficiency?

Answer 30

Iatrogenic long-term steroid use

Question 31

In secondary adrenal insufficiency there is loss of which hormones?

Answer 31

Glucocorticoids only

Question 32

Is hyperpigmentation seen in those with secondary adrenal insufficiency? Why/why not?

Answer 32

No - ACTH is low in these cases

Question 33

How is a diagnosis of congenital adrenal hyperplasia confirmed?

Answer 33

Increased level of 17-hydroxyprogesterone