Acute cortisol insufficiency

Question 1

What is the alternative name for primary adrenal insufficiency?

Answer 1

Addison disease

Question 2

What are 4 things that can cause Addison disease in childhood?

Answer 2

1. Autoimmune process
2. Haemorrhage/infarction - neonatal, meningococcal sepsis
3. X-linked adrenleucodystrophy
4. Tuberculosis

Question 3

What are 3 things that primary adrenal insufficiency can be associated with when the cause is an autoimmune process?

Answer 3

other autoimmune endocrine disorders e.g.

1. Diabetes mellitus
2. Hypothyroidism
3. Hypoparathyroidism

Question 4

What is X-linked adrenlolecuodystrophy?

Answer 4

rare neurodegenerative metabolic disorder

Question 5

What are 2 things that adrenal insufficiency can be secondary to (unlike primary adrenal insufficiency)?

Question 6

What are 7 features of the acute presentation of adrenal insufficiency?

Answer 6

1. Hyponatraemia
2. Hyperkalaemia
3. Hypoglycaemia
4. Dehydration
5. Hypotension
6. Growth failure
7. Circulatory collapse

Question 7

What are 3 chronic features of adrenal insufficiency?

Answer 7

1. Vomiting
2. Lethargy
3. Brown pigmentation (gums, scars, skin creases)

Question 8

How is a diagnosis of adrenal insufficiency made? 6 key aspects

Answer 8

• finding hyponatraemia and hyperkalaemia
• often associated with metabolic acidosis and hypoglycaemia
• plasma cortisol is low and plasma ACTH concentration high (except in pituitary dysfunction/exogenous steroids)

Question 9

What test can be performed to help in the diagnosis of adrenal insufficiency? What is a positive result for adrenal insufficiency?

Answer 9

ACTH (synacthen) test - plasma cortisol concentrations remain low in both primary adrenal dysfunction and long-standing pituitary/hypothalamic dysfunction

Question 10

What does a normal response to the short synacthen test indicate?

Answer 10

It excludes adrenal insufficiency

Question 11

What are the 3 immediate treatments of adrenal insufficiency (adrenal crisis)?

Answer 11

1. IV saline
2. IV glucose
3. IV hydrocortisone

Question 12

What are the 2 long-term treatments of adrenal insufficiency?

Answer 12

1. Glucocorticoid
2. Mineralocorticoid

Question 13

When must the dose of long-term glucocorticoid be changed when treating adrenal insufficiency?

Answer 13

Must be increased by three times at times of illness or for an operation

Question 14

What emergency treatment for adrenal insufficiency must parents be taught how to use?

Answer 14

Taught how to inject intramuscular hydrocortisone in an emergency

Question 15

What must children at risk of adrenal crisis have to show their risk?

Answer 15

MedicAlert brace or necklace, and carry a steroid card

Question 16

What are the 3 key causes of adrenal insufficiency to remember?

Answer 16

1. Long-term corticosteroid therapy
2. Congenital adrenal hyperplasia
3. Addison’s disease (more rare)