Adrenal steroids

Question 1

All congenital adrenal enzyme deficiencies characterized by

Answer 1

Enlargement of bilateral adrenal glands

due to high ACTH stimulation from low cortisol

Question 2

17-alpha hydroxylase deficiency:

lab

Answer 2

Mineralocorticoid: high
Cortisol: low
Sex hormone: low

Question 3

17-alpha hydroxylase deficiency:

presentation

Answer 3

HTN, Hypokalemia

XY: low DTH so pseudohermaphroditism; variable, ambiguous genitalis, undescended testes

XX: externally phenotypic female with normal internal sex organs, lacks 2nd sex characteristics

Question 4

21-hydroxylase deficiency:

lab

Answer 4

Mineralocorticoid: low
Cortisol: low
Sex hormone: high

Question 5

21-hydroxylase deficiency:

presentation

Answer 5

Most common form

HYPOtension, hypoerkalemia, high renin activity, low vol.

Masculinzation, leading to pseudohermaphroditism in female

Question 6

11-hydroxylase deficiency:

lab

Answer 6

Mineralocorticoid: low aldo, high 11-deoxycorticosterone
Cortisol: low
Sex hormone: high

Question 7

11-hydroxylase deficiency:

presentation

Answer 7

HTN (11-deoxycorticosterone is a minralocorticoid and secreted in excess)

Masculinzation

Question 8

Angiotensin II affects

Answer 8

Induces aldosterone synthase,

which converts corticosterone to aldosterone