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19 Cards in this Set

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Bicornate Uterus
**Results from incomplete fusion of the paramesonephric ducts

**Associated w/ URINARY TRACT abnormalities and infertility
Congenital Penile Abnormalities
1) Hypospadias
2) Epispadias
Hypospadias
**Abnormal opening of the penile urethra on the INFERIOR (ventral) side of the penis

**Due to failure of the urethral folds to close
Epispadias
**Abnormal opening of the penile urethra on superior (dorsal) side of the penis due to faulty positioning of the genital tubercle

**Exstrophy of the bladder is associated with epispadias
Which is more common?
HYPOSPADIAS are more common than epispadias

**You want to fix hypospadias to prevent UTIs
Klinefelter's Syndrome
**47XXY
=male
=1:850

**Presence of an inactivated X-chromosome (=Barr body)

IMAGE 107
Features:
=Testicular atrophy
=Eunuchoid body shape
=Tall, long extremities
=Gynecomastia
=Female hair distribution

**Common cause of hypogonadism seen in infertility workup
Turner's Syndrome
**45XO
=female
=1:3000
Features:
=Short stature
=Ovarian dysgenesis (streak ovary)
=Webbing of the neck
=Coarcation of hte aorta

**MOST COMMON CAUSE of PRIMARY amenorrhea

**NO barr body

COLOR IMAGE 108
Double Y Males
47XYY
=male
=1:1000

**Observed w/ increased frequency amoung inmates of penal institutions
Features:
=Phenotypically normal
=Very tall
=Severe acne
=Antisocial behavior --> seen in 1-2% of XYY males
Psudohermaphroditism
**Disagreement between the phenotypic (external genitalia) and gonadal (testes vs. ovaries) sex
Female Pseudohermaphrodite
**XX**

**Ovaries are present, but external genitalia are VIRILIZED or ambiguous
Cause?
**Due to excessive and inappropriate exposure to androgenic steroids during early gestation --> i.e.

=CAH
=Exogenous administration of androgens during pregnancy
Male Pseudohermaphrodite
**XY**

**Testes present, but external genitalia are FEMALE or ambiguous
Cause:
**Most common form is ANDROGEN INSENSITIVITY SYNDROME
=testicular feminization
True Hermaphrodite
**46XX or 47XXY

**Both ovary and testicular tissue present
=ambiguous genitalia

**VERY rare
Androgen Insensitivity Syndrome
46XY

**Defect in androgen receptor resulting in NORMAL-appearing female
=female external genitalia w/ rudimentary vagina
=uterus and uterine tubes generally absent
=develops testes--> surgically removed to prevent malignancy
Hormone Profile?
=Increased levels of testosterone, estrogen, and LH