Endocrinology: Addison Disease

Question 1

Is it rare in childhood?

Answer 1

Yes

Question 2

What can it result from?

Answer 2

Autoimmune process (sometimes in association with DM, hypothyroidism, hypoparathyroidism)
Haemorrhage/infarction - e.g in neonatal sepsis
X linked adrenoleucodystrophy (rare neurodegenerative metabolic disorder)
TB

Question 3

Adrenal insufficiency may be secondary due to…

Answer 3

Pituitary dysfunction - from hypothalamus-pituitary disease or from HPA suppression from long term corticosteroids

Question 4

Do infants usually present acutely or not?

Answer 4

Usually acutely

Question 5

Describe the presentation

Answer 5

Hyponatraemia
Hyperkalaemia
Hypoglycaemia 
Dehydration
Hypotension
Growth failure
Circulatory collapse

Question 6

In older children, presentation is usually with non specific symptoms such as…

Answer 6

Fatigue
Brown pigmentation - gums, scars, skin creases
Vomiting
Postural hypotension - check lying and standing BP

Question 7

How is it diagnosed?

Answer 7

Hyponatraemia 
Hypokalaemia
Metabolic acidosis 
Hypoglycaemia 
The plasma cortisol is low and the plasma ACTH is high (except in pituitary dysfunction) 
ACTH synacthen test

Question 8

What is the synacthen test?

Answer 8

Plasma cortisol taken before and after synthetic ACTH given

- absent cortisol response = hypoaldrenalism

Question 9

What can precipitate an Addisonian crisis?

Answer 9

Severe stress, salt deprivation, infection, abrupt steroid withdrawal

Question 10

What are the symptoms of Addisonian crisis?

Answer 10

Hypotension
Tachycardia
Pyrexia
Nausea and vomiting

Question 11

How is an adrenal crisis managed?

Answer 11

IV saline and glucose

IV hydrocortisone