Congenital Adrenal Hyperplasia

Question 1

What electrolye abnormalities would be seen in an adrenal crisis?

Answer 1

Low sodium
High potassium

Question 2

What is the pathophysiology of CAH?

Answer 2

21-hydroxylase deficiency leads to high androgens, but low cortisol and aldosterone

Question 3

What blood gas picture can an adrenal crisis cause?

Answer 3

Metabolic acidosis

Question 4

What is the acute treatment of an adrenal crisis in CAH?

Answer 4

High dose hydrocortisone
Dextrose
Sodium chloride
IV fluids

Question 5

Why is dextrose given in an adrenal crisis?

Answer 5

Blood sugar will be low in an adrenal crisis

Question 6

How do boys present with CAH?

Answer 6

Boys often present with an adrenal crisis shortly after birth

Question 7

How do girls present with CAH?

Answer 7

Girls typically present at birth with virilised genitalia

Question 8

What symptoms may girls present with in puberty?

Answer 8

Excess hair growth
Tall for their age
Deep voice
Early puberty
Absent periods

Question 9

Is antenatal treatment for CAH possible?

Answer 9

Treatment with dexamethasone is possible in mothers carrying girls. Dexamethasone is started in all mothers suspected of carrying a girl with CAH, and is continued until term if the fetus is a girl

Question 10

What is the treatment of CAH?

Answer 10

Hydrocortisone
Fludrocortisone

Question 11

What happens to the treatment of CAH when the patient is ill?

Answer 11

SIck day rules apply and steroids need to be increased