Congenital Adrenal Hyperplasia Flashcards Preview

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Flashcards in Congenital Adrenal Hyperplasia Deck (12):
1

hyponatremic hyperkalemic, metabolic acidosis indicates ______

mineralocorticoid deficiency --> adrenal crisis/salt-wasting crisis

2

Tx for salt wasting crisis

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

3

how does sugar reduce potassium?

induces insulin

4

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

21 hydroxylase deficient congenital adrenal hyperplasia

5

CAH

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

6

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

T --> even though all are made ineffiecient

7

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

reduced, increased, reduced

8

Mineralocorticoid excess/deficiency causes hypertension and hypokalemia

excess

9

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

deficiency

10

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

T

11

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

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

12

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

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