Congenital Adrenal Hyperplasia Flashcards Preview

Endocrine > Congenital Adrenal Hyperplasia > Flashcards

Flashcards in Congenital Adrenal Hyperplasia Deck (12)
Loading flashcards...
1
Q

hyponatremic hyperkalemic, metabolic acidosis indicates ______

A

mineralocorticoid deficiency –> adrenal crisis/salt-wasting crisis

2
Q

Tx for salt wasting crisis

A

iv isotonic saline, dextrose to lower potassium, high dose hydrocortisone to provide vascular support and slow Na loss

3
Q

how does sugar reduce potassium?

A

induces insulin

4
Q

leading diagnosis of salt-wasting crisis in first weeks of life

A

21 hydroxylase deficient congenital adrenal hyperplasia

5
Q

CAH

A

inborn error of cortisol synthesis that result in compensatory high ACTH resulting in adrenal hyperplasia in fetal life and beyond

6
Q

T/F most clinical manifestations of CAH are caused by excess/deficiency of mineralocorticoids and sex hormones vs by glucocorticoids

A

T –> even though all are made ineffiecient

7
Q

21OH impairment leads to reduced/increased mineralocorticoid production, reduced/increased androgen production, and reduced/increased cortisol production.

A

reduced, increased, reduced

8
Q

Mineralocorticoid excess/deficiency causes hypertension and hypokalemia

A

excess

9
Q

Mineralocorticoid excess/deficiency causes saltwasting, hyponatremia, hyperkalemia, dehydration

A

deficiency

10
Q

T/F effects of mineralocorticoid excess/deficiency are independent of sex.

A

T

11
Q

Why are there about 20% more adult females than males with CAH?

A

harder to identify boys b/c testosterone doesn’t result in clinical features –> die from saltwasting without dx of CAH

12
Q

T/F moderate 21OH CAH would result in saltwasting in XY individual

A

F –> but would cause precocious puberty, overgrowth, early pubic hair