DISORDERS OF ADRENAL CORTEX

Question 1

WHAT IS ADDISON’S DISEASE?

Answer 1

• primary adrenal insufficiency - hypoadrenalism characterised by reduced cortisol and aldosterone

Question 2

WHAT ARE THE CAUSES OF ADDISON’S DISEASE?

Answer 2

1) autoimmune destruction of adrenal gland

2) genetic defects in steroid synthesis

Question 3

WHAT ARE THE SYMPTOMS OF ADDISON’S DISEASE?

Answer 3

1) fatigue
2) muscle weakness
3) anorexia
4) weight loss
5) nausea
6) abdominal pain

Question 4

WHAT ARE THE SIGNS OF ADDISON’S DISEASE?

Answer 4

1) Glucocorticoid loss - hypoglycaemia, increased pigmentation (ACTH excess due to reduced negative feedback)
2) Androgen loss - reduced libido and loss of axillary and pubic hair
3) Mineralocorticoid deficiency - postural hypotension and dizziness

Question 5

WHAT ARE THE INVESTIGATIONS FOR ADDISON’S DISEASE?

Answer 5

1) Serum electrolytes
2) Blood urea
3) FBC
4) Morning serum cortisol - between 8am and 9am when cortisol levels are usually at its highest
5) Serum ACTH
6) Synacthen test

Question 6

WHAT ARE THE BIOCHEMCIAL HALLMARKS FOR ADDISON’S DISEASE?

Answer 6

1) hyponatraemia
2) hyperkalaemia
3) hypoglycaemia
4) raised urea
5) mild anaemia

Question 7

WHAT ARE THE CORTISOL AND ACTH LEVELS IN ADDISON’S DISEASE?

Answer 7

• low 9am cortisol and simultaneously raised ACTH concentration.

Question 8

WHAT IS A SYNACTHEN TEST AND WHY IS IT USED?

Answer 8

• Synacthen is a synthetic copy of ACTH, when given adrenal glands should respond by releasing cortisol.
• Blood sample taken and tested for cortisol before synacthen injection. Further blood samples to measure cortisol after 30 and 60 minutes.
• Results- increase ACTH but low cortisol and aldosterone
• Used to confirm Addison’s disease following positive blood results.

Question 9

WHAT IS THE PHARMACOLOGICAL MANAGEMENT FOR PATIENTS WITH ADDISON’S DISEASE?

Answer 9

Lifelong corticosteroid replacement therapy

1) HYDROCORTISONE - replace cortisol, administration should resemble natural cycle of corticosteroid release therefore larger dose in morning and smaller in evening. Alternative is low dose prednisolone (less common)
2) FLUDROCORTISONE- replace aldosterone
3) Androgen replacement is not routinely prescribed

Question 10

WHAT SHOULD PATIENTS WITH ADDISON’S DISEASE DO DURING PERIODS OF ILLNESS AND WHY?

Answer 10

• patients should double their glucocorticoid dose at times of illness and continue on doubled dose until illness has resolved.
• this is because when ill (or when exercising), patient is under physical stress and increase risk of adrenal crisis.
• adrenal glands cannot supply extra corticosteroids to cope with stress

Question 11

WHAT IS AN ADDISONS CRISIS AND WHAT CAUSES IT?

Answer 11

• Life threatening state caused by insufficient levels of cortisol
• Caused by severe physical stress such as severe infection, trauma

Question 12

WHAT ARE THE SYMPTOMS OF ADDISON CRISIS?

Answer 12

1) Extreme weakness
2) Extremely low blood pressure
3) Sweating
4) Loss of consciousness
5) Increase heart rate
6) Dizziness
7) Confusion

Question 13

WHAT IS THE TREATMENT FOR ADDISON CRISIS?

Answer 13

• IV/ IM hydrocortisone

- IV fluids due to low BP

Question 14

WHAT ARE ADDISON PATIENTS REQUIRED TO CARRY?

Answer 14

• steroid emergency card
• medical alert jewellery
• emergency contact details for endocrine team

Question 15

WHAT SELF-CARE ADVICE IS GIVEN FOR ADDISON PATIENTS?

Answer 15

• aware that they require life long corticosteroid replacement therapy
• aware of symptoms of Addison crisis
• aware of how to administer IM hydrocortisone (family member should be aware aswell).

Question 16

WHAT IS THE CAUSE OF SECONDARY ADRENAL INSUFFICIENCY (ACTH DEFCIENCY)?

Answer 16

• hypopituitarism - pituitary gland doesn’t make ACTH therefore no cortisol release

Question 17

WHAT IS THE PHARMACOLOGICAL TREATMENT OF SECONDARY ADRENAL INSUFFICIENCY?

Answer 17

1) HYDROCORTISONE

Question 18

WHY IS FLUDROCORTIONE REPLACMENT NOT REQUIRED IN SECODNARY ADRENAL INSUFFICIENCY?

Answer 18

• mineralocorticoid is not required as aldosterone is regulated by RAAS.

Question 19

WHY SHOULD PATIENTS NOT STOP THEIR STEORIDS ABRUPTLY?

Answer 19

• needs to be tapered off because prolonged therapy with corticosteroids causes adrenal atrophy (as adrenal gland not producing hormones itself). Abrupt withdrawal after a prolonged period can lead to acute adrenal insufficiency, hypotension, or death.