021615 congenital anomalies of GU tract Flashcards Preview

ER part 2 > 021615 congenital anomalies of GU tract > Flashcards

Flashcards in 021615 congenital anomalies of GU tract Deck (10)
Loading flashcards...
1
Q

features of undervirilized males

A
small phallus
hypospadias
cryptorchidism
bifid scrotum
absence of scrotal rugation
2
Q

features of virilized female

A

clitoromegaly
common UG sinus
fused labioscrotal folds
rugated labioscrotal folds

3
Q

most common cause of 46 XX DSD

A

21 hydroxylase deficiency (testos and DHT are increased)

4
Q

50% of cases of 46XY are due to

A

idiopathic

5
Q

ovotesticular DSD

A

both ovarian and testicular tissue with normal responsiveness to hormones

karyotype: 46XX, 46XY, 46XX/XY

fertility is uncommon

6
Q

complete androgen insensitivity syndrome-problem is mainly for

A

males (XY)

7
Q

what would you see in puberty for complete androgen insensit syndrome

A

feminizing puberty without menses (breast development b/c of excess testosterone)

8
Q

DHT is critical for

A

virilization prior to birth (testos is critical at puberty)

9
Q

in CAH, what amts of androgens are produced?

A

no testicular androgens but excessive adrenal androgens are produced

10
Q

why do Wolffian ducts regress in CAH in 46 XX?

A

b/c there’s no excess LOCAL androgens