Deficiency of Adrenal Hormones

Question 1

What is the most common cause of adrenal insufficiency?

What is the most common cause in children?

Answer 1

Addison’s disease (autoimmune disease)

CAH - congenital adrenal hyperplasia

Question 2

What is adrenal insufficiency?

Answer 2

Can’t produce enough cortisol/aldosterone

Question 3

How does adrenal insufficiency present?

What is the buzzword symptom for Addison’s?
Explain why this symptom occurs and hence why it wouldn’t occur in secondary causes of AI?

Answer 3

Very generically

• Fatigue
• Weight loss/anorexia
• Dizziness/postural hypotension (primary causes only)
• abdo pain, N+V

“tanned”/”hyperpigmentation”
- occurs mainly in folds of skin/inside of mouth

Increase in ACTH (no negative feedback) activates melanocytes to produce more melanin

Secondary causes of AI = low ACTH = pale skin

Question 4

What are your investigation results for adrenal insufficiency?
Explain them

Answer 4

Low cortisol - reduce blood sugar

Low aldosterone (in primary causes)

• reduced BP
• low Na+/high K+

Question 5

What autoantibody is associated with Addison’s?

Answer 5

21-OH (21-hydroxylase)

Question 6

Name 2 secondary causes of adrenal insufficency?

What is the commonest?

Answer 6

Pituitary gland adenoma etc -> reduced ACTH production

Commonest = sudden withdrawl of exogenous steroid use

Question 7

If suspect adrenal insufficiency wait until diagnostic test before treating. T/F?

Answer 7

F

Question 8

How is adrenal insufficiency managed?

Answer 8

IV hydrocortisone initally
-> hydrocortisone 2-3 times daily
FLUIDS

may need Fludrocortisone to increase aldosterone levels (if primary)

Manage and conselling around sick day rules and carrying steroid ID card

Question 9

What is the emergency associated with adrenal insufficiency?

How does it present?

How is it managed?

Answer 9

Adrenal crisis

Reduced consciousness
Hypotension
Hypoglycaemia, hyponatraemia, hyperkaemia
Patients can be very unwell

100mg IV hydrocortisone (repeat every 6 hrs)
Fluids
Close monitoring

Question 10

What diagnostic test is done to test for adrenal insufficiency?

What is the criteria for having it

Answer 10

SynACTHen test

Failure of cortisol to raise above 550nmol/l = AI

Question 11

What is the difference in the biochem of primary and secondary AI?

Answer 11

Primary - ACTH high

Secondary

• ACTH low
• aldosterone normal

Question 12

Where is the genetic mutation often found in CAH?

What does this lead to?

Answer 12

21alpha-hydroxylase

Increase in testosterone + decrease in aldosterone and cortisol

Question 13

CAH classically presents in children. How does it present?

In the non-classical presentation, it will present normally in older females. How do they present?

Answer 13

Classical

• salt-wasting (decrease in aldosterone = loads of salt loss)
• virilisation (ambiguous genitalia in females)

Non-classical (assoc. with increase in testosterone)

• acne
• hiritsim
• infertility/sub-fertility
• precious puberty
• oligomenorrhoea (infrequent periods)

Question 14

How is CAH diagnosed?

Answer 14

Basal 17-OH progesterone levels

Genetic testing

Question 15

How is CAH managed?

Answer 15

Simillary to Addison’s

• hydrocortisone
• mineralcorticoid treatment in some (Fludrocortisone)

Question 16

How should you manage a patient who is acutely unwell who is taking glucocorticoids?

Answer 16

Double dosage for duration of illness

ONLY glucocorticoid not mineralocorticoid

Question 17

How is a patient taken hydrocortisone for addison’s told to take it?

Answer 17

Split over 2/3 doses with maj. taken in the morning