Adrenal Insufficiency

Question 1

Adrenal insufficiency is when the adrenal glands don’t produce enough steroid hormones, particularly ____ and ____

Answer 1

cortisol and aldosterone

Question 2

What is cortisol used for?

Answer 2

Helps regulate metabolism, blood pressure and body’s response to stress

Question 3

What disease refers to damaged adrenal glands (primary adrenal insufficiency) resulting in reduced secretion of cortisol and aldosterone?

Answer 3

Addison’s disease

Question 4

What is the most common cause of Addison’s disease in the developed world?

Answer 4

autoimmunity

Question 5

What is the most common cause of adrenal insufficiency in the developing world?

Answer 5

TB
(also sarcoidosis)

Question 6

What antibodies are present in Addison’s disease?

Answer 6

21-alpha-hydroxylase auto antibodies

Question 7

What is Waterhouse-Friderichsen syndrome?

Answer 7

Adrenal insufficiency due to adrenal haemorrhage, often due to meningococcal meningitis

Question 8

What causes secondary adrenal insufficiency?

Answer 8

Main cause is iatrogenic (steroids)
Also by adrenal metastasis (lungs, liver, breast) or adrenal haemorrhage

Inadequate ACTH stimulating the adrenal glands resulting in low levels of cortisol being released. Loss or damage of pituitary gland. HPA suppression.

Question 9

What can damage the pituitary gland?

Answer 9

congenital underdevelopment (hypoplasia)
Surgery
Infection
Loss of blood flow
Radiotherapy

Question 10

True or false: secondary adrenal insufficiency can cause hyperpigmentation

Answer 10

False - No ACTH to stimulate the melanocytes. Happens for primary adrenal insufficiency

Question 11

Tertiary adrenal insufficiency is the result of inadequate ___ release by the _____

Answer 11

CRH, hypothalamus

Question 12

What can cause tertiary adrenal insufficiency?

Answer 12

Long term (more than 3 weeks) oral steroids causing suppression of hypothalamus.

When the exogenous steroids are suddenly withdrawn, the hypothalamus doesn’t kick into action fast enough and endogenous steroids are not adequately produced.

Question 13

What should be tapered slowly to allow time for the adrenal axis to regain normal function?

Answer 13

Long term steroids

Question 14

What are features of adrenal insufficiency?

Answer 14

Lethargy
Weight loss
Postural hypotension (when stand up)
Vitiligo
Hyperpigmentation (primary)
Change in body hair
Abdominal pain
Vomiting
Hypoglycaemia

In babies also:
Poor feeding
Jaundice
Failure to thrive

Question 15

What are features of adrenal insufficiency in older children?

Answer 15

Nausea and vomiting
Poor weight gain or weight loss
Reduced appetite (anorexia)
Abdominal pain
Muscle weakness
Muscle cramps
Developmental delay
Poor academic performance
Bronze hyperpigmentation to skin in Addison’s (high ACTH levels stimulating melanocytes)

Question 16

What investigations are made for suspected adrenal insufficiency?

Answer 16

U&Es:
Hyponatraemia and hyperkalaemia

Blood glucose:
Hypoglycaemia

Test levels of:
Cortisol, ACTH, aldosterone and renin

Short synacthen Test (ACTH stimulation test)

CXR is TB suspected

Question 17

What is the short synacthen test?

Answer 17

Used to confirm adrenal insufficiency
Done in morning, 9am as cortisol highest.
Involves giving synacthen (synthetic ACTH). Then blood cortisol measured at baseline, 30mins and 60 mins post administration.
The cortisol levels should at least double in response to synacthen. If not, indicates primary adrenal insufficiency.

Question 18

How do you manage adrenal insufficiency?

Answer 18

Replacement steroid titrated to signs, symptoms and electrolytes.

Cortisol is replaced with the glucocorticoid hormone Hydrocortisone.

Aldosterone is replaced with the mineralocorticoid hormone Fludrocortisone.

Patients given steroid card and emergency ID tag

Question 19

Why are patients given steroid card and emergency ID tag?

Answer 19

They are dependent on their steroids for life. Doses are increased during an acute illness to match normal steroid response to illness.

Question 20

What is an adrenal crisis?

Answer 20

Also called Addisonian crisis, emergency that occurs due to severe deficiency of cortisol.
Often triggered by stress, illness, surgery, trauma or interruption of corticosteroid management, increasing the body’s demand for cortisol.

Question 21

What are signs and symptoms of adrenal crisis?

Answer 21

Low blood pressure
Hyponatremia
Hyperkalaemia
Severe weakness
Fatigue
Nausea
Vomiting
Abdominal pain
Dehydration
Confusion
Loss of consciousness