Adrenal steroid synthesis

Question 1

Fetal adrenal gland

Answer 1

Helps mature type II pneumocytes w/ cortisol secretion

Question 2

Adrenal cortex

Answer 2

Mesoderm
MC benign adrenal adeoma site

GFR (outer to inner)
Zona Glomerulosa – salt, aldosterone
Zona fasciculate – sugar, glucocorticoid
Zona Reticularis – sex, androgens

“Deeper you go, sweeter it gets”

Question 3

Adrenal medulla

Answer 3

Chromaffin cells – neural crest

Produce catecholamines – Epi/NE

MC adrenal medulla tumor in adults: pheochromocytoma

• HA, sweats, severe HTN, tachycardia
• episodic

MC adrenal medulla tumor in kids:
Adrenal neuroblastoma
-sustained HTN

Question 4

Congenital adrenal hyperplasia

Answer 4

Deficient 21 a hydroxylase

No aldosterone, no cortisol all shuttled to testosterone

-> masculinization of girls, salt wasting in boys (and girls)

hypotension, hyponatremia, hyperkalemia

labs: marked elevation of 17-OH progesterone

Question 5

3-beta-hydroxysteroid dehydrogenase deficiency

Answer 5

Inability to produce glucocorticoids, mineralocorticoids, androgens, estrogen

Excessive sodium excretion in urine

Early death

Question 6

17a hydroxylase deficiency

Answer 6

Inability to produce sex hormones and cortisol -> phenotypic female unable to mature

Increased production of mineralocorticoids -> sodium and fluid retention -> HTN

Question 7

21 a – hydroxylase deficiency

Answer 7

Inability to produce cortisol -> elevated ACTH
Inability to produce mineralocorticoids -> hypotension, salt wasting
Increased production of sex hormones -> masculinization

Question 8

11 b hydroxylase deficiency

Answer 8

Inability to produce cortisol, corticosterone, aldosterone

Increased production of deoxycorticosterone (weak mineralocorticoid) -> HTN

Incrased production of sex hormones -> masculinization