CAH Flashcards
In normal sexual differentiation, Wolffian duct development is caused by what hormone?
Testosterone
In normal sexual differentiation, formation of male external genitalia is caused by what hormone?
DHT
21 hydroxylase deficiency causes the following EXCEPT
a. salt-wasting
b. low cortisol
c. low 17-OHP
d. high androgen
e. high ACTH
C
True of CAH EXCEPT
a. females are more commonly affected
b. most common is 21-hydroxylase deficiency
c. Autosmal recessive
d. NOTA
A
Classic vs. Non-classic 21-OH deficiency EXCEPT
a. Classic 21-OH deficiency could be salt-losing or simple virilizing form
b. Non-classic 21-OH deficiency has normal cortisol and normal aldosterone
c. masculinization of genitalia is common in classic and non-classic
d. in non-classic 21-OH deficiency, there is normal 17-OHP
D
True of salt-losing 21-OHdef CAH
a. high androgens
b. hyperglycemia
d. high cortisol
d. high aldosterone
A; no cortisol- hypoglycemia; no aldosterone; increased cortisol precursors; increased androgens
True of Simple virilizing 21-OHdef CAH EXCEPT
a. normal cortisol
b. increased aldosterone
c. increased androgens
d. low 17-OHP
D;
True of Non-classic 21-OH deficiency EXCEPT
a. normal cortisol
b. normalaldosterone
c. normal 17-OHP
d. increased androgens
C
TRUE of classic 21-OH def
a. males are identified later in childhood because of precocious pubic hair, penile enlargement
b. in simple virilizing form, females are identified earlier in life because of failure to thrive
c. Hyperpigmentation of the genitalia is due to increased ACTH
d. NOTA
e. AOTA
C; males identified earlier because of salt-wasting
virilizing - females identified later for precocious pubic hair and/or clitoromegaly; hyperpigmentation: increased ACTH stimulate melanocortin receptor
Short term medical management of 21-OH def CAH include the ff EXCEPT
a. IV bolus isotonic NaCl 20ml/kg
b. IV bolus isotonic NaCl 450 ml/m2
c. All classical CAH patients should be treated with fludrocortisone acetate at diagnosis
d. Hydrocortisone at 10-15mg/m2/dose
D. Hydrocortisone at 10-15mg/m2/day 3 divided doses
MOA of corticotrohin releasing hormone antagonist
reduce adrenal hyperstimulation
MOA of carbenoxolone
increase levels of bioavailable cortisol
What should be done in CAH patient when medical management is failing?
Bilateral adrenelectomy
Surgical treatment: The goals of surgery include the ff EXCEPT
a. genital appearance compatible with gender
b. unobstructed urinary emptying without incontinence or infections
c. good adult sexual and reproductive function
d. NOTA
e. AOTA
D
