Congenital Adrenal Hyperplasia and other things! Flashcards Preview

STEP1 > Congenital Adrenal Hyperplasia and other things! > Flashcards

Flashcards in Congenital Adrenal Hyperplasia and other things! Deck (17):
1

Embryological origin of the medulla and cortex of the adrenal gland?

medulla-ectoderm
cortex-mesoderm

2

What is the most common tumor of the adrenal medulla in adults? Children?

Adults--pheochromocytoma
Children-neuroblastoma

3

What is the most common location for an adrenal gland tumor?

cortex

4

Which type of HTN is produced by pheochromocytoma v. neuroblastoma

pheochromocytoma--episodic HTN
neuroblastoma--HTN rare, when present-->sustained

5

How does 17-alpha hydroxylase deficiency present in men? Women?

Men: ambiguous genitalia, undescended testes
Women: delayed secondary sexual characteristics

6

What is the lab finding with 17-alpha hydroxylase deficiency?

Decreased androstenedione

7

What is the presentation of 21 hydroxylase deficiency in men and women?

Men: salt wasting, precocious puberty
Women: virilization

8

What is the lab finding in 21 hydroxylase deficiency?

increase in 17-hydroxyprogesterone

9

Which substance is critical for the development of type II pneumocytes in utero? At which gestational age?

34 weeks
cortisol

10

What are the features of 21 alpha hydroxylase deficiency?

Decreased aldosterone, cortisol
Increased androgens
Get hypotension and hyperkalemia
Get masculinization
get increased 17hydroxyprogesterone (directly upstream,screening test)

11

What are the features of 11beta hydroxylase deficiency?

Decreased aldosterone, cortisol
Increased DOC (mineralcorticoid-like)
Increased androgens
HTN
Masculinization

12

What are the features of 17alpha hydroxylase deficiency?

Decreased androgens
Increased aldosterone and decreased cortisol
Ambiguous genitalia, or lack of secondary sex characteristics in women
HTN, hypokalemia.

13

THe lack of _____ in congenital adrenal hyperplasia (all forms) causes an increase in ____ which results in hyperplasia of the adrenal gland.

lack of cortisol
ACTH increases

14

What does ketoconazole inhibit in the steroid synthesis pathway?

desmolase
Cholesterol-->Pregnenolone

15

What does Ang II stimulate in the steroid synthesis pathway?

aldosterone synthase

16

What does ACTH stimulate in the steroid synthesis pathway?

desmolase
end up with more aldosterone and cortisol etc.

17

What does finasteride do to the steroid synthesis pathway?

inhibits 5alpha reductase
get decreased DHT, helps w/ male pattern baldness and BPH